Our mission is to advance mitochondrial research and to develop diagnostic tools for mitochondrial disease and drug-induced mitochondrial toxicity.

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3 About MitoSciences Our mission is to advance mitochondrial research and to develop diagnostic tools for mitochondrial disease and drug-induced mitochondrial toxicity. We offer the world s largest library of mitochondrial monoclonal antibodies made to exacting standards of specificity and utility backed by leading researchers. We also offer a range of kits that employ our mitochondrial antibodies in highly useful formats for achieving a wide range of research goals. Additionally, we manufacture all of our monoclonal antibodies and research kits, we use them in our own research, and all of our products are 100% guaranteed.

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5 Table of Contents Products by Protein...2 Complex I...3 Complex II...5 Complex III...7 Complex IV...9 Complex V...13 AIF (Apoptosis-Inducing Factor)...15 ANT...23 Cyclophilin D...19 Cytochrome c...19 Frataxin...21 Mitochondrial Trifunctional Protein (MTP)...23 Mitofilin...25 PDH...27 Porin...29 Products by Research Focus...31 Mitochondrial Genetic Diseases...33 Apoptosis...35 Oxidative Stress...37 Cancer...39 Alzheimer's Disease...41 Parkinson's Disease...45 Reference Products...48 Ancillary Products...52 Tables Species Reactivity by Product...54

6 Products by Protein

7 Complex I Complex I, or NADH ubiquinone oxidoreductase, is a large protein complex (950,000 MW) consisting of different subunits. A total of 7 of the subunits of the complex are encoded on mtdna, the remainder is nuclear-encoded, made in the cytosol and translocated into the organelle for assembly at the inner membrane. The enzyme complex catalyses electron entry from NADH, as the name implies, via a flavin (FMN) and several non-heme iron centers. Complex I is sensitive to a wide range of inhibitors, many of which, such as rotenone, are pesticides or other common environmental toxins. Complex I dysfunction is a common cause of genetic OXPHOS defects. Altered functioning of this complex is also thought to contribute to several neurological disorders including Parkinson s disease and schizophrenia. Additionally, there is evidence of Complex I involvement in diabetes. Immunoassays MS101 MitoProfile Complex I Immunocapture Kit 250 g, 500 g or 750 g monoclonal antibody irreversibly crosslinked to protein G-agarose beads which can immunocapture ~60 g, ~120 g or ~180 g respectively of Complex I from heart mitochondria. Dipstick Assays MS130 MitoProfile Dipstick Assay for Complex I Activity Contains 30 or 60 dipsticks and necessary components for quantitative analysis of NADH activity in human, bovine, rat, and mouse samples. MS131 MitoProfile Dipstick Assay for Human Complex I Quantity Contains 30 or 60 dipsticks and necessary components for quantitative analysis of NADH levels in human and bovine samples. MS133 MitoProfile Dipstick Assay for Rodent Complex I Quantity Contains 30 or 60 dipsticks and necessary components for quantitative analysis of NADH levels in mouse and rat samples. Monoclonal Antibodies MS101c Complex I Immunocapture mab 100 g monoclonal antibody which can immunocapture ~25 g of Complex I from heart mitochondria. MS103 Complex I subunit GRIM-19 mab 100 g of monoclonal antibody reactive against the 19 kda Complex I subunit GRIM-19. MS103 also labels GRIM-19 in immunocytochemistry applications. MS104 Complex I subunit NDUFS4 mab 100 g of monoclonal antibody reactive against the 22 kda Complex I subunit NDUFS4. 3

8 MS105 Complex I subunit NDUFB8 mab 100 g of monoclonal antibody reactive against the 20kDa Complex I (NADH dehydrogenase) subunit NDUFB8. Formerly listed as likely subunit ND6. MS107 Complex I subunit NDUFB4 mab 100 g of monoclonal antibody reactive against the 15 kda Complex I (NADH dehydrogenase) subunit NDUFB4. MS107 also labels in immunocytochemical applications. Formerly listed as likely subunit NDUFA6. MS108 Complex I subunit NDUFB6 mab 100 g of monoclonal antibody reactive against the 20 kda Complex I subunit NDUFB6. MS109 Complex I subunit 8 kda mab 100 g of monoclonal antibody reactive against an 8 kda Complex I subunit. MS110 Complex I subunit NDUFS3 mab 100 g of monoclonal antibody reactive against the 30 kda Complex I subunit NDUFS3. MS110 also labels NDUFS3 in immunocytochemistry applications. MS111 Complex I subunit NDUFA9 mab 100 g of monoclonal antibody reactive against the 39 kda Complex I subunit NDUFA9. MS112 Complex I subunit NDUFS3 mab 100 g of monoclonal antibody reactive against the 30 kda Complex I subunit NDUFS3 in humans and C. elegans.

9 Complex II Complex II is also called succinate ubiquinone oxidoreductase, or more commonly succinate dehydrogenase complex. This complex is composed of 4 subunits and contains a flavin (FAD), non-heme iron centers, and a b-type cytochrome as prosthetic groups. It is both a component of the electron transport chain and an enzyme of the Krebs cycle. Complex II deficiencies are seen in OXPHOS genetic disease and found in a type of cancer called paraganglioma. Immunoassays MS201 MitoProfile Complex II Immunocapture Kit 250 g, 500 g or 750 g monoclonal antibodies irreversibly crosslinked to protein G- agarose beads which can immunocapture ~13 g, ~26 g or ~39 g respectively of Complex II from heart mitochondria. MS202 MitoProfile Human Complex II Cocktail Contains a total of 300 g or 600 g of antibodies and is a pre-mixed solution of 3 monoclonal antibodies against Complex II subunits 70 kda Fp, 30 kda Ip along with an antibody against Complex V subunit as a control. This cocktail can be used to verify isolated Complex II purity or to conveniently determine the levels of 2 of the 4 Complex II subunits in patient cell lines relative to the control protein (Complex V subunit ). Monoclonal Antibodies MS201c Complex II Immunocapture mab 100 g monoclonal antibody which can immunocapture ~5 g of Complex II from heart mitochondria. MS203 Complex II subunit 30 kda Ip mab 100 g of monoclonal antibody reactive against the Complex II 30 kda Ip subunit in Western blotting and immunocytochemistry applications. MS204 Complex II subunit 70 kda Fp mab 100 g of monoclonal antibody reactive against the Complex II 70 kda Fp subunit in Western blotting and immunocytochemistry applications. 5

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11 Complex III Complex III, or ubiquinol cytochrome c oxidoreductase catalyses electron transfer from reduced ubiquinone or coenzyme Q 10 to cytochrome c. The complex contains 11 different subunits, one of which, cytochrome b, is encoded on mtdna, while the remainder are nuclear-encoded. Genetic Complex III deficiencies occur but are rare. Immunoassays MS301 MitoProfile Complex III Immunocapture Kit 250 g, 500 g or 750 g monoclonal antibodies irreversibly crosslinked to protein G- agarose beads which can immunocapture ~25 g, ~50 g or ~75 g respectively of Complex III from heart mitochondria. Monoclonal Antibodies MS301c Complex III Immunocapture mab 100 g monoclonal antibody which can immunocapture ~10 g of Complex III from heart mitochondria. MS303 Complex III subunit Core 1 mab 100 g of monoclonal antibody reactive against the 49 kda complex III protein core 1. MS304 Complex III subunit Core 2 mab 100 g of monoclonal antibody reactive against the 47 kda Complex III protein core 2. MS304 also labels core 2 in immunocytochemistry applications. MS305 Complex III subunit Rieske mab 100 g of monoclonal antibody reactive against the 22 kda complex III Rieske subunit. MS306 Complex III subunit 10 kda mab 100 g of monoclonal antibody reactive against a complex III 10 kda subunit. 7

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13 Complex IV Complex IV, also called cytochrome c oxidoreductase or cytochrome c oxidase, is a complex of 13 different subunits, 3 of which (I, II and III) are encoded on mtdna, with the remainder nuclear-encoded. The complex contains 2 heme groups (a and a 3 ) and 2 copper atoms as prosthetic groups. Genetic alterations of this enzyme complex are a common cause of OXPHOS diseases, and the enzyme is altered in Alzheimer s disease patients. Also, there are reports of reduced amounts of this complex in cancer cells when these cells become hypoxic. Immunoassays MS401 MitoProfile Complex IV Immunocapture Kit 250 g, 500 g or 750 g monoclonal antibodies irreversibly crosslinked to protein G- agarose beads which can immunocapture ~25 g, ~50 g or ~75 g respectively of Complex IV from heart mitochondria. MS402 MitoProfile Complex IV Immunocapture Kit for Rodent 250 g, 500 g or 750 g monoclonal antibodies irreversibly crosslinked to protein G- agarose beads which can immunocapture ~25 g, ~50 g or ~75 g respectively of Complex IV from heart mitochondria. The Complex IV immunocapture kit allows isolation of the cytochrome c oxidase complex (EC ) from small amounts of rat or mouse tissue. MS441 MitoProfile Rapid Microplate Assay Kit for Human Complex IV Activity A rapid activity microplate kit that is used to determine the activity of cytochrome c oxidase in a human sample with greater speed and simplicity. Includes buffers, detergent, substrates, and 96-well microplate with monoclonal antibody pre-bound to the wells of the plate, allowing for a stream-lined assay. MS442 MitoProfile Rapid Microplate Assay Kit for Human Complex IV Quantity A rapid activity microplate kit that is used to determine the amount of cytochrome c oxidase in a human sample with greater speed and simplicity. Includes buffers, detergent, substrates, and 96-well microplate with monoclonal antibody pre-bound to the wells of the plate, allowing for a stream-lined assay. MS443 MitoProfile Rapid Microplate Assay Kit for Human Complex IV Activity and Quantity (Multiplexing) A 96-well multiplexing microplate kit that is used to determine both the activity and quantity of cytochrome c oxidase in a human sample with greater speed and simplicity. Includes buffers, detergent, substrates, and 96-well microplate with monoclonal antibody pre-bound to the wells of the plate, allowing for a stream-lined assay. MS444 MitoProfile Rapid Microplate Assay Kit for Mouse Complex IV Activity A 96-well activity microplate kit that is used to determine the activity of cytochrome c oxidase in a mouse sample with greater speed and simplicity. Includes buffers, detergent, substrates, and 96-well microplate with monoclonal antibody pre-bound to the wells of the plate, allowing for a stream-lined assay. 9

14 MS445 MitoProfile Rapid Microplate Assay Kit for Mouse Complex IV Quantity A 96-well activity microplate kit that is used to determine the amount of cytochrome c oxidase in a mouse sample with greater speed and simplicity. Includes buffers, detergent, substrates, and 96-well microplate with monoclonal antibody pre-bound to the wells of the plate, allowing for a stream-lined assay. MS446 MitoProfile Rapid Microplate Assay Kit for Mouse Complex IV Activity and Quantity (Multiplexing) A 96-well multiplexing microplate kit that is used to determine both the activity and quantity of cytochrome c oxidase in a mouse sample with greater speed and simplicity. Includes buffers, detergent, substrates, and 96-well microplate with monoclonal antibody pre-bound to the wells of the plate, allowing for a stream-lined assay. MS447 MitoProfile Rapid Microplate Assay Kit for Rat Complex IV Activity A 96-well activity microplate kit that is used to determine the activity of cytochrome c oxidase in a rat sample with greater speed and simplicity. Includes buffers, detergent, substrates, and 96-well microplate with monoclonal antibody pre-bound to the wells of the plate, allowing for a stream-lined assay. Dipstick Assays MS430 MitoProfile Dipstick Assay Kit for Human Complex IV Activity Contains 30 or 60 dipsticks and necessary components for quantitative analysis of cytochrome c oxidase activity in human and bovine samples. MS431 MitoProfile Dipstick Assay Kit for Human Complex IV Quantity Contains 30 or 60 dipsticks and necessary components for quantitative analysis of cytochrome c oxidase levels in human and bovine samples. MS432 MitoProfile Dipstick Assay Kit for Rodent Complex IV Activity Contains 30 or 60 dipsticks and necessary components for quantitative analysis of cytochrome c oxidase activity in mouse and rat samples. Monoclonal Antibodies MS401c Complex IV Immunocapture mab 100 g monoclonal antibody which can immunocapture ~10 g of Complex IV from heart mitochondria. MS403 Complex IV subunit SURF1 mab 100 g of monoclonal antibody reactive against the 33 kda Complex IV assembly factor SURF1. Cellular levels of SURF1 are highest in growing cultured cells. MS404 Complex IV subunit I mab 100 g of monoclonal antibody reactive against the 57 kda Complex IV subunit I. The apparent molecular weight is 35 kda. MS404 also labels COX I in immunocytochemistry applications.

15 MS405 Complex IV subunit II mab 100 g of monoclonal antibody reactive against the 26 kda Complex IV subunit II. MS405 also labels COX II in immunocytochemistry applications. MS406 Complex IV subunit III mab 100 g of monoclonal antibody reactive against the 30 kda Complex IV subunit III. The apparent molecular weight is 22 kda. MS407 Complex IV subunit IV mab 100 g of monoclonal antibody reactive against the 18 kda Complex IV subunit IV. Reacts with human, bovine, mouse and rat. MS408 also binds COX IV. MS408 (alternate to 407) Complex IV subunit IV mab 100 g of monoclonal antibody reactive against the 18 kda Complex IV subunit IV. MS408 also labels COX IV in Immunocytochemistry applications. MS407 also binds COX IV. Reacts only with human and bovine, but better than MS407 for immunocytochemistry in these species. MS409 Complex IV subunit Va mab 100 g of monoclonal antibody reactive against the 12 kda Complex IV subunit Va. MS409 also labels COX Va in immunocytochemistry applications. MS410 Complex IV subunit Vb mab 100 g of monoclonal antibody reactive against the 13 kda Complex IV subunit Vb. MS411 Complex IV subunit VIaH mab 250 g of monoclonal antibody reactive against the 12 kda Complex IV subunit VIa and specific to the heart isoform. MS412 Complex IV subunit VIaL mab 250 g of monoclonal antibody reactive against the 12 kda Complex IV subunit VIa and specific to the 'liver' isoform. MS413 Complex IV subunit VIb mab 100 g of monoclonal antibody reactive against the 12 kda Complex IV subunit VIb. MS414 Complex IV subunit Vlc mab 100 g of monoclonal antibody reactive against the 12 kda Complex IV subunit VIc. MS414 also labels COX VIc in immunocytochemistry applications. MS415 Complex IV subunit VIIaH & VIIaL mab 250 g of monoclonal antibody reactive against the 8 kda Complex IV subunit VIIa and reacting against both liver and heart isoforms. MS416 Complex IV subunit VIIb mab 250 g of monoclonal antibody reactive against the 8 kda Complex IV subunit VIIb. MS418 Complex IV subunit I (COX1 protein) mab for yeast 100 g of monoclonal antibody reactive against the COX (Complex IV) subunit I in Saccharomyces cerevisiae for use in Western blotting applications. 11

16 MS419 Complex IV subunit II (COX2 protein) mab for yeast 100 g of monoclonal antibody reactive against the COX (Complex IV) subunit II in Saccharomyces cerevisiae for use in Western blotting applications. MS420 Complex IV subunit IV (COX4 protein) mab for yeast 100 g of monoclonal antibody reactive against the COX (Complex IV) subunit IV in Saccharomyces cerevisiae for use in Western blotting applications.

17 Complex V Complex V, also called F 1 F 0 ATPase and ATP Synthase, is responsible for ATP production in oxidative phosphorylation and can work in reverse as a proton pumping ATPase. The enzyme was thought to be localized exclusively to mitochondria but it has recently been identified also on the plasma membrane of several cell types including hepatocytes where it is the HDL receptor, on endothelial cells where it may act as the angiostatin receptor, and on the surface of cancer cells. The enzyme in mammals is composed of 17 subunits, 5 of which make up the easily detached F 1. The remainder are components of 2 stalk domains and the proton pumping F 0 part of the machinery. Two of the subunits of the F 0 part are encoded on mtdna while the other subunits are nuclear-encoded. Mutations in the mitochondrial encoded subunits of Complex V cause OXPHOS disease. Immunoassays MS501 MitoProfile Complex V Immunocapture Kit 250 g, 500 g or 750 g of monoclonal antibodies irreversibly crosslinked to protein G- agarose beads which can immunocapture ~125 g, ~250 g or ~500 g respectively of Complex V from heart mitochondria. MS541 MitoProfile Rapid Microplate Assay Kit for ATP Synthase (Complex V) Activity A rapid activity microplate kit that is used to determine the activity of ATP synthase in human or rat samples with greater speed and simplicity. Includes buffers, detergent, lipid mix, and 96-well microplate with monoclonal antibody pre-bound to the wells of the plate, allowing for a stream-lined assay. MS542 MitoProfile Rapid Microplate Assay Kit for ATP Synthase (Complex V) Quantity A rapid activity microplate kit that is used to determine the amount of ATP synthase in human or rat samples with greater speed and simplicity. Includes buffers, detergent, lipid mix, and 96-well microplate with monoclonal antibody pre-bound to the wells of the plate, allowing for a stream-lined assay. MS543 MitoProfile Rapid Microplate Assay Kit for ATP Synthase (Complex V) Activity and Quantity (Multiplexing) A 96-well multiplexing microplate kit that is used to determine both the activity and quantity of ATP synthase in human or rat samples with greater speed and simplicity. Includes buffers, detergent, lipid mix, and 96-well microplate with monoclonal antibody pre-bound to the wells of the plate, allowing for a stream-lined assay. Monoclonal Antibodies MS501c Complex V Immunocapture mab 100 g monoclonal antibody which can immunocapture up to 50 g of Complex V from heart mitochondria. 13

18 MS502 Complex V subunit mab 100 g of monoclonal antibody reactive against the 55 kda F 1 F 0 ATP synthase, Complex V (alpha) subunit. MS502 also labels the subunit in immunocytochemistry applications. MS503 Complex V subunit mab 100 g of monoclonal antibody reactive against the 52 kda F 1 F 0 ATP synthase, Complex V (beta) subunit. MS503 also labels the subunit in immunocytochemistry applications. MS504 Complex V subunit mab 100 g of monoclonal antibody reactive against the 19 kda F 1 F 0 ATP synthase, Complex V (delta) subunit. MS504 also labels the d subunit in immunocytochemistry applications. MS505 Complex V subunit OSCP mab 100 g of monoclonal antibody reactive against the 21 kda F 1 F 0 ATP synthase, Complex V OSCP subunit. MS505 also labels the OSCP subunit in immunocytochemistry applications. MS506 Complex V IF 1 subunit mab 100 g of monoclonal antibody reactive against the 9.6 kda Complex V IF1 subunit. The antibody shows in Western blotting of whole mitochondria shows reactivity with two bands at 10 kda and at 18 kda, but in isolated F 1 F 0 only the band at 10 kda is observed (IF1). The 18 kda band is the subunit IV of Complex IV. MS507 Complex V mab with broad cross-reactivity 100 g of monoclonal antibody reactive against the 55 kda F 1 F 0 ATP synthase, Complex V (alpha) subunit. This mab has broad species cross-reactivity including animal and plant subunits. MS507 also labels the subunit in immunocytochemistry applications. MS508 Complex V F 6 subunit mab 100 g of monoclonal antibody reactive against the 9 kda F 1 F 0 ATP synthase, Complex V F 6 subunit. MS508 also labels the 6 subunit in immunocytochemistry applications. MS511 Complex V F 1 -F 0 -beta subunit mab 100 g of monoclonal antibody reactive against the 9 kda F 1 F 0 ATP synthase (Complex V) beta subunit.

19 AIF (Apoptosis-Inducing Factor) AIF is a flavoprotein (67 kda) localized in the inter-membrane space. It is a multi-functional protein with a vital oxidoreductase function (required for cell survival), an anti-oxidant activity, an assembly function in Complex I (NADH-ubiquinone oxidoreductase complex) formation and an apoptogenic function. AIF has a major role in the caspase-independent apoptosis process. It translocates to the nucleus after mitochondrial release, leading to chromatin condensation and DNA fragmentation. Dipstick Assays MSA31 MitoProfile Dipstick Assay Kit for Apoptosis-Inducing Factor (AIF) Contains 30 or 60 dipsticks and necessary components to quantify the amount of Apoptosis- Inducing Factor (AIF) from human sample material. Monoclonal Antibodies MSA09 AIF (Apoptosis-Inducing Factor) mab 100 g of monoclonal antibody reactive against the 67 kda AIF, suitable for use in for Western blotting and immunocytochemistry applications. 15

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21 ANT ANT is a reversible transporter of ATP and ADP. During OXPHOS the protein exchanges ATP out for ADP in, but when it becomes important to maintain a membrane potential, at times when OXPHOS is not working, ANT can work in reverse. Mutant ANT has been identified in cardiomyopathy, and autoimmune reactions to this protein also cause heart related diseases. Immunoassays MSA01 MitoProfile ANT Immunocapture Kit 250 g monoclonal antibody irreversibly crosslinked to protein G-agarose beads which can immunocapture ANT from heart mitochondria. Monoclonal Antibodies MSA01c ANT immunocapture mab 100 g monoclonal antibody which can immunocapture ANT from heart mitochondria. MSA02 ANT mab 100 g of monoclonal antibody reactive against the 33 kda adenine nucleotide translocase (ANT). MSA02 also labels ANT in immunocytochemistry applications. 17

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23 Cyclophilin D Cyclophilin D is the binding site for cyclosporins and this protein may gate the PTP. Monoclonal Antibodies MSA04 Cyclophilin D mab 100 g of monoclonal antibody reactive against the 22 kda human Cyclophilin D (P30405). MSA04 also labels Cyclophilin D in immunocytochemistry applications. 19

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25 Cytochrome c Cytochrome c is a water-soluble mitochondrial intermembrane-space protein loosely attached to the inner mitochondrial membrane. It is approximately 13 kda and it plays important roles in oxidative phosphorylation and apoptosis. As an electron carrier in oxidative phosphorylation cytochrome c shuttles four electrons, one at time, via its heme group from Complex III (cytochrome c reductase) to Complex IV (cytochrome c oxidase). As a crucial player in apoptosis (programmed cell death) cytochrome c is released from the mitochondria to the cytosol where it binds to an adaptor subunit, APAF-1 in the presence of datp, leading to activation of caspase 9. Caspase 9 triggers activation of other caspases, which cleave and destroy other proteins. This results in cell death. Immunoassays MSA07 ApoTrack Cytochrome c Apoptosis Kit for Immunocytochemistry MSA07 detects apoptotic cells with high specificity. It contains an anti-cytochrome c monoclonal antibody (50 g) and the appropriate isotype-specific secondary antibody (50 g) which has a fluorescein fluorophore conjugated to it. The kit also contains an anti-complex V monoclonal antibody (50 g) and the appropriate Texas Red fluorophore-conjugated isotype-specific secondary antibody (50 g). Complex V serves as a mitochondrial marker in healthy and apoptotic cells. MSA11 ApoTrack Cytochrome c Apoptosis Kit for Western blotting Contains 180 g of premixed cocktail of 4 monoclonal antibodies and necessary buffers and detergent allowing for a rapid method to measure the fraction of cytochrome c released from mitochondria into the cytosol during apoptosis. Human heart mitochondria are included as a Western blotting control. MSA12 ApoTrack Cytochrome c Apoptosis Cocktail Contains 180 g of premixed cocktail of 4 monoclonal antibodies that allows detection of cytochrome c in the cytoplasmic and mitochondria-containing fractions by Western Blot analysis to determine the proportion of released cytochrome c from mitochondria to cytoplasm. Human heart mitochondria are also included as a Western blotting control. MSA41 MitoProfile Rapid Microplate Assay Kit for Cytochrome c Quantity A 96-well activity microplate kit that is used to determine the amount of cytochrome c in a human, bovine, mouse, or rat samples. Includes detergent, substrates, and 96-well microplate with monoclonal antibody pre-bound to the wells of the plate, allowing for a stream-lined assay. Monoclonal Antibodies MSA06 Cytochrome c mab 100 g of monoclonal antibody against cytochrome c, suitable for use in Western blotting and immunocytochemistry. 21

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27 Frataxin Frataxin is a nuclear encoded mitochondrial protein. In humans the gene is located on chromosome 9. Friedreich s Ataxia (FRDA) is an inherited recessive neurodegenerative disorder caused by decreased expression levels of frataxin. In the majority of patients having FRDA, the codon GAA is repeated times in the first intron of both copies of the gene. Frataxin has been implicated in mitochondrial iron homeostasis; however the mechanism is still unknown. Deletion of the frataxin gene in yeast results in iron accumulation in mitochondria and loss of respiration. Increased mitochondrial iron levels have also been observed in patients with FRDA. In addition, recombinant human frataxin has been shown to bind iron in-vitro. Dipstick Assays MSF31 MitoProfile Dipstick Assay Kit for Frataxin Contains 30 or 60 dipsticks and necessary components for quantitative analysis of human frataxin protein levels. Monoclonal Antibodies MSF01 Frataxin mab 100 g of monoclonal antibody against frataxin, suitable for use in Western blotting and Immunocytochemistry. 23

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29 Mitochondrial Trifunctional Protein (MTP) Mitochondrial Trifunctional Protein (MTP) is part of the mitochondrial fatty acid beta-oxidation pathway. It is located in the inner mitochondrial membrane and it catalyzes three out of the four steps in the beta-oxidation cycle with a specific affinity for long chain substrates. MTP is hetero-octamer composed of four and four -subunits. The -subunit is a 79 kda enzyme which has two enzymatic functions, the hydration of enoyl-coa (E.C ) and the dehydrogenation of 3-hydroxyacyl CoA (LCHAD - E.C ). The -subunit, a 47 kda enzyme, performs the third function - the thiolytic cleavage of 3-ketoacyl-CoA substrates (E.C ). Inherited deficiency of MTP is a recessive genetic disease which occurs in approximately 1:38,000 newborns in the US. Deficiency of LCHAD (the dehydrogenase part of MTP) has also been found in children of women who develop HELLP syndrome (1:1,000 pregnancies) and AFLP syndrome (1:13,000 pregnancies), both of which are life-threatening obstetric conditions. Furthermore, heterozygous individuals for MTP defects are more susceptible to nonalcoholic hepatic steatosis, or nonalcoholic fatty liver disease (NAFLD), the most common form of liver disease in the US with a recorded prevalence as high as 39%. Immunoassays MS740 MitoProfile Rapid Microplate Assay Kit for Human Mitochondrial Trifunctional Protein (MTP) A 96-well plate assay for rapidly determining the assembled enzyme quantity of MTP in a human sample. Dipstick Assays MS740 MitoProfile Dipstick Assay Kit for Human Mitochondrial Trifunctional Protein (MTP) Contains 30 or 60 dipsticks and necessary components to quantify the amount of the Mitochondrial Trifunctional Protein (MTP) from human sample material. 25

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31 Mitofilin Mitofilin is a transmembrane protein of the inner mitochondrial membrane and is associated with a large multimeric protein complex of about 1200 kda. Mitofilin has critical functions in mitochondrial morphology and mitochondrial fusion and fission, specifically in the formation of tubular cristae and cristae junctions. Abnormal mitochondrial morphology has been implicated in many human diseases such as myopathy, cardiomyopathy, rhabdomyosarcoma and Whartin's tumor. Monoclonal Antibodies MSM02 Mitofilin mab 100 g of monoclonal antibody against mitofilin, suitable for use in Western blotting and immunocytochemistry. The mitofilin monoclonal antibody reacts against 2 isoforms (about 90 and 91kDa) in Western blots with human heart mitochondria. A single form of mitofilin is detected in Western blots with human liver mitochondria. 27

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33 PDH The pyruvate dehydrogenase complex (PDH) is at the centre of aerobic carbohydrate metabolism. It is localized in the matrix space of mitochondria where it catalyzes the irreversible oxidative decarboxylation of pyruvate entering the organelle to produce acetyl- CoA, NADH and CO 2. PDH is a complex of 3 different enzymes that channel substrate and product. These are pyruvate dehydrogenase (E1), dihydrolipoamide transacetylase (E2) and dihydrolipoamide dehydrogenase (E3). Pyruvate dehydrogenase is a heterotetramer of two alpha and two beta subunits and the activity of this enzyme is regulated by a specific E1 kinase and phospho-e1-phosphatase, which respectively phosphorylate and dephosphorylate the enzyme. Phosphorylation inactivates the enzyme which is reversed by the phosphatase reaction. The total PDH complex has a MW of over 8 million and is composed of 60 copies of E1, 60 copies of E2 and 12 copies of E3 along with 12 copies of E3BP, a protein required for interaction of E3 with E2. PDH complex deficiency is a relatively common cause of metabolic and neurodegenerative disorders. Enzyme defects are thought to contribute to late-onset diabetes and the activity of the enzyme is altered by diet and exercise. Also PDH deficiency has been found in patients with sepsis. Immunoassays MSP01 MitoProfile PDH Immunocapture Kit 250 g monoclonal antibody irreversibly crosslinked to protein G-agarose beads which can immunocapture ~13 g of PDH complex from heart mitochondria. MSP02 MitoProfile PDH Western Blotting Kit Contains a total of 150 g or 300 g respectively of monoclonal antibodies reactive against PDH subunits. MSP18 MitoProfile Rapid Microplate Assay Kit for PDH Activity A rapid activity microplate kit that is used to determine the activity of PDH in a human, bovine, mouse, or rat sample with greater speed and simplicity. Includes buffer, detergent, reagent mix, and 96-well microplate with monoclonal antibody pre-bound to the wells of the plate, allowing for a stream-lined assay. This kit is optimized for use with tissue extract when the amount of total material available for assay is g or more. MSP19 MitoProfile Rapid Microplate Assay Kit for PDH Quantity A rapid activity microplate kit that is used to determine the amount of PDH in a human, bovine, mouse, or rat sample with greater speed and simplicity. Includes buffers, detergent, substrates, and 96-well microplate with monoclonal antibody pre-bound to the wells of the plate, allowing for a stream-lined assay. MSP20 MitoProfile Rapid Microplate Assay Kit for PDH Activity and Quantity (Combo) A rapid combination of MSP18 and MSP19 microplate assay kits that are used to determine both the activity and quantity of PDH in a human, bovine, mouse, or rat sample with greater speed and simplicity. Includes buffers, detergent, reagent mix, substrates, and 96-well microplate with monoclonal antibody pre-bound to the wells of the plate, allowing for a stream-lined assay. 29

34 Dipstick Assays MSP30 MitoProfile Dipstick Assay Kit for PDH Activity Contains 30 or 60 dipsticks and necessary components for quantitative analysis of PDH activity in human, bovine, mouse, and rat samples. MSP31 MitoProfile Dipstick Assay Kit for PDH Quantity Contains 30 or 60 dipsticks and necessary components for quantitative analysis of PDH levels in human, bovine, mouse, and rat samples. Monoclonal Antibodies MSP01c PDH Immunocapture mab 100 g monoclonal antibody which can immunocapture PDH from heart mitochondria. MSP03 PDH subunit E1 mab 100 g of monoclonal antibody reactive against the 44 kda PDH E1 subunit. MSP03 also labels E1 in immunocytochemistry applications. MSP04 PDH subunit E1 mab 100 g of monoclonal antibody reactive against the 39 kda PDH subunit E1. MSP05 PDH subunit E2 mab 100 g of monoclonal antibody reactive against the 66 kda PDH E2 subunit. MSP05 also labels E2 in immunocytochemistry applications. MSP06 PDH subunit E2/E3bp mab 100 g of monoclonal antibody reactive against both the 66 kda PDH E2 subunit and the 54 kda E3 binding protein. Preliminary data suggest that this antibody recognizes bound lipoic acid. MSP06 also labels E2 & E3bp in immunocytochemistry applications. MSP07 PDH subunit E1 mab 100 g of monoclonal antibody reactive against the 44 kda PDH E1 subunit. MSP07 also labels E1 in immunocytochemistry applications.

35 Porin The channel-forming protein porin (also called the voltage dependent anion channel, VDAC) is involved in both aerobic metabolism and apoptotic processes. Porin is localized in the outer membrane of mitochondria where it forms the primary pathway of metabolites across the membrane. Porin can also serve as a binding site for enzymes or a regulatory molecule. Monoclonal Antibodies MSA03 Porin mab 100 g of monoclonal antibody reactive against the 31 kda human porin (VDAC1) for Western blotting applications. MSA05 Porin mab 100 g of monoclonal antibody reactive against human porin (VDAC1) for immunocytochemistry. MSA08 Porin mab for yeast 100 g of monoclonal antibody reactive against Porin in Saccharomyces cerevisiae for use in Western blotting applications. 31

36 Products by Research Focus

37 Mitochondrial Genetic Diseases Genetic diseases affecting Oxidative Phosphorylation (OXPHOS) occur in around 1 in 7,000 live births and are also found amongst the adult population. Analysis of these diseases remains problematic. They can occur through mutations in mtdna and also by mutation of the many proteins encoded on nuclear DNA and transported to the mitochondrion to complete the OXPHOS complexes. Presently, initial detection of a likely OXPHOS dysfunction is made by activity measurements of individual OXPHOS complexes as well as ATP levels. While such analyses are useful, they are difficult, tedious and require significant amounts of cell culture material or biopsy tissue. Moreover, there is considerable overlap between measurements of activities for individuals with clear disease and the ranges of the same activity found in the general population. Further, activity measurements are proving difficult to standardize between laboratories. MitoSciences has a protein analysis approach to defining OXPHOS dysfunction which uses antibodies to detect reduced levels of key subunits of each of the OXPHOS complexes and the pyruvate dehydrogenase complex in as few as one plate of cells and from needle biopsy amounts of tissue. The protein analysis tests are fast, reliable and easy to perform as each is provided in kit form. Immunoassays MS601 MitoProfile Human Total OXPHOS Complexes Detection Kit Contains a total of 360 g or 720 g of antibodies and is a premixed solution of 5 monoclonal antibodies against a subunit of each of the 5 OXPHOS complexes. Human heart mitochondria are also included as a Western blotting control. MS602 MitoProfile OXPHOS/PDH Immunocytochemistry Kit A cocktail of 220 g antibodies comprised of six monoclonal antibodies packaged individually. Each antibody is against a labile subunit of each of the 5 OXPHOS complexes and PDH. Each mab is provided in a 50 l aliquot at a 200x concentration, to allow reconstitution of each mab in 10 ml working volume. MS602a MitoProfile OXPHOS/PDH Immunocytochemistry Kit Includes the same components as MS602 (above), but additionally contains 60 g mab to porin, and 120 g to Complex V subunit, and the required secondary antibodies for simultaneous 2-dye staining of samples. MS603 MitoProfile Blue Native OXPHOS Complexes Detection Kit A cocktail of monoclonal antibodies which allows the rapid and highly sensitive detection of OXPHOS components from very small sample amounts. This cocktail allows the simultaneous analysis of the assembly state of all 5 OXPHOS complexes from a single patient sample. MS604 MitoProfile Rodent Total OXPHOS Complexes Detection Kit Contains a total of 300 g or 600 g of antibodies and is a premixed solution of 5 monoclonal antibodies against a subunit of each of the 5 OXPHOS complexes in mitochondrial preparations from mouse sources. 33

38 MS740 MitoProfile Rapid Microplate Assay Kit for Human Mitochondrial Trifunctional Protein (MTP) A 96-well plate assay for rapidly determining the assembled enzyme quantity of MTP in a human sample. Dipstick Assays MS740 MitoProfile Dipstick Assay Kit for Human Mitochondrial Trifunctional Protein (MTP) Contains 30 or 60 dipsticks and necessary components to quantify the amount of the Mitochondrial Trifunctional Protein (MTP) from human sample material.

39 Apoptosis A number of proteins found in mitochondria combine under circumstances favoring apoptosis to form the so-called permeability transition pore. Proteins variously identified as a part of the PTP include cytosolic hexokinase, outer membrane porin (also called the voltage dependent anion channel or VDAC), adenylate cyclase in the intermembrane space, the adenine nucleotide transporter (ANT) and the peripheral benzodiazepine receptor protein of the inner membrane along with cyclophilin D of the matrix space. Hexokinase, porin and ANT all occur as isoforms and it remains to be determined whether there is specificity of these different forms for the PTP. Immunoassays MSA07 ApoTrack Cytochrome c Apoptosis Kit for Immunocytochemistry MSA07 detects apoptotic cells with high specificity. It contains an anti-cytochrome c monoclonal antibody (50 g) and the appropriate isotype-specific secondary antibody (50 g) which has a fluorescein fluorophore conjugated to it. The kit also contains an anti-complex V monoclonal antibody (50 g) and the appropriate Texas Red fluorophore-conjugated isotype-specific secondary antibody (50 g). Complex V serves as a mitochondrial marker in healthy and apoptotic cells. MSA11 ApoTrack Cytochrome c Apoptosis Kit for Western blotting Contains 180 g of premixed cocktail of 4 monoclonal antibodies and necessary buffers and detergent allowing for a rapid method to measure the fraction of cytochrome c released from mitochondria into the cytosol during apoptosis. Human heart mitochondria are included as a Western blotting control. MSA12 ApoTrack Cytochrome c Apoptosis Cocktail Contains 180 g of premixed cocktail of 4 monoclonal antibodies that allows detection of cytochrome c in the cytoplasmic and mitochondria-containing fractions by Western Blot analysis to determine the proportion of released cytochrome c from mitochondria to cytoplasm. Human heart mitochondria are also included as a Western blotting control. MSA41 MitoProfile Rapid Microplate Assay Kit for Cytochrome c Quantity A 96-well activity microplate kit that is used to determine the amount of cytochrome c in a human, bovine, mouse, or rat samples. Includes detergent, substrates, and 96-well microplate with monoclonal antibody pre-bound to the wells of the plate, allowing for a stream-lined assay. Dipstick Assays MSA31 MitoProfile Dipstick Assay Kit for Apoptosis-Inducing Factor (AIF) Contains 30 or 60 dipsticks and necessary components to quantify the amount of Apoptosis- Inducing Factor (AIF) from human sample material. 35

40 Monoclonal Antibodies MSA01c ANT Immunocapture mab 100 g monoclonal antibody which can immunocapture ANT from heart mitochondria. MSA02 ANT mab 100 g of monoclonal antibody reactive against the 32 kda adenine nucleotide translocase (ANT). MSA02 also labels ANT in immunocytochemistry applications. MSA03 Porin mab 100 g of monoclonal antibody reactive against the 39 kda human porin (VDAC1) for Western blotting applications. MSA04 Cyclophilin D mab 100 g of monoclonal antibody reactive against the 21 kda human Cyclophilin D (P30405). MSA04 also labels Cyclophilin D in immunocytochemistry applications. MSA05 Porin mab 100 g of monoclonal antibody reactive against the 39 kda human porin (VDAC1) for immunocytochemistry. MSA06 Cytochrome c mab 100 g of monoclonal antibody against the 12 kda cytochrome c, suitable for use in Western blotting and Immunocytochemistry. MSA08 Porin monoclonal antibody for yeast 100 g of monoclonal antibody reactive against Porin in Saccharomyces cerevisiae for use in Western blotting applications. MSA09 AIF (Apoptosis-Inducing Factor) mab 100 g of monoclonal antibody reactive against the 67 kda AIF, suitable for use in for Western blotting and immunocytochemistry applications.

41 Oxidative Stress Mitochondria are a primary site of production of free radicals. While more than 98% of the molecular oxygen taken up by cells is fully utilized by cytochrome c oxidase to form water, this enzyme can release partly reduced species. Other enzymes of the respiratory chain, and in particular complexes I and III, also produce partly reduced oxygen species including superoxide. These reactive oxygen species can react with nitric oxide to produce reactive nitrogen species including peroxynitrite. While a significant proportion of the reactive oxygen and nitrogen species diffuse into the cytosol, a major portion remains in the mitochondrion. The levels of reactive oxygen and nitrogen species produced is a function of the activity and more specifically the dysfunction of the OXPHOS chain. The less efficient electron transfer is, the more radicals are produced. Free radical damage of cytosolic proteins increases with increased levels of radical production. Moreover, oxidative and nitrative damage of mitochondrial proteins adds to OXPHOS dysfunction further exacerbating free radical production. Enhanced oxidative stress is now well documented to occur in a number of degenerative diseases including Parkinson s disease, Alzheimer s and diabetes. MitoSciences has a set of mabs that can immunocapture all 5 OXPHOS complexes for subsequent analysis of accrued oxidative and nitrative damage, either with antibodies to the modified amino acid or by mass spectrometric analysis. Immunoassays MS101 MitoProfile Complex I Immunocapture Kit 250 g, 500 g or 750 g monoclonal antibody irreversibly crosslinked to protein G-agarose beads which can immunocapture ~60 g, ~120 g or ~180 g respectively of Complex I (NADH dehydrogenase) from heart mitochondria. MS201 MitoProfile Complex II Immunocapture Kit 250 g, 500 g or 750 g monoclonal antibodies irreversibly crosslinked to protein G- agarose beads which can immunocapture ~13 g, ~26 g or ~39 g respectively of Complex II from heart mitochondria. MS301 MitoProfile Complex III Immunocapture Kit 250 g, 500 g or 750 g monoclonal antibodies irreversibly crosslinked to protein G- agarose beads which can immunocapture ~25 g, ~50 g or ~75 g respectively of Complex III from heart mitochondria. MS401 MitoProfile Complex IV Immunocapture Kit 250 g, 500 g or 750 g monoclonal antibodies irreversibly crosslinked to protein G- agarose beads which can immunocapture ~25 g, ~50 g or ~75 g respectively of Complex IV from heart mitochondria. MS501 MitoProfile Complex V Immunocapture Kit 250 g, 500 g or 750 g of monoclonal antibodies irreversibly crosslinked to protein G- agarose beads which can immunocapture ~25 g, ~50 g or ~75 g respectively of Complex V from heart mitochondria. 37

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43 Cancer Mitochondrial involvement in cancer is at several levels. Genetic alterations in OXPHOS genes can predispose to cancer, as in the case of succinate dehydrogenase subunit mutations, which cause paraganglioma syndromes. Also, metabolic changes in energy metabolism are characteristic of cancer as tumors switch from an OXPHOS mode to glycolysis as source of ATP production. Further, the organelle is a key part of the signaling in apoptosis, a process that must be down-regulated to allow cell proliferation in the disease. Finally, recent work indicates that there is recruitment of mitochondrial proteins to the plasma membrane of cancer cells. Immunoassays MS201 MitoProfile Complex II Immunocapture Kit 250 g, 500 g or 750 g monoclonal antibodies irreversibly crosslinked to protein G- agarose beads which can immunocapture ~13 g, ~26 g or ~39 g respectively of Complex II from heart mitochondria. MS501 MitoProfile Complex V Immunocapture Kit 250 g, 500 g or 750 g of monoclonal antibodies irreversibly crosslinked to protein G- agarose beads which can immunocapture ~25 g, ~50 g or ~75 g respectively of Complex V from heart mitochondria. MS601 MitoProfile Human Total OXPHOS Complexes Detection Kit Contains a total of 360 g or 720 g of antibodies and is a premixed solution of 5 monoclonal antibodies against a subunit of each of the 5 OXPHOS complexes in mitochondrial preparations from human sources. Human heart mitochondria are also included as a Western blotting control. MS604 MitoProfile Rodent Total OXPHOS Complexes Detection Kit Contains a total of 300 g or 600 g of antibodies and is a premixed solution of 5 monoclonal antibodies against a subunit of each of the 5 OXPHOS complexes in mitochondrial preparations from mouse sources. MSA41 MitoProfile Rapid Microplate Assay Kit for Cytochrome c Quantity 96-well activity microplate kit that is used to determine the amount of cytochrome c in a human, bovine, mouse, or rat samples. Includes detergent, substrates, and 96-well microplate with monoclonal antibody pre-bound to the wells of the plate, allowing for a stream-lined assay. Monoclonal Antibodies MS404 Complex IV subunit I mab 100 g of monoclonal antibody reactive against the COX (Complex IV) subunit I. MS404 also labels COX I in immunocytochemistry applications. MS407 Complex IV subunit IV mab 100 g of monoclonal antibody reactive against the COX (Complex IV) subunit IV. MS408 also binds to COX IV. 39

44 MS502 Complex V subunit mab 100 g of monoclonal antibody reactive against the F 1 F 0 ATP synthase (Complex V) (alpha) subunit. MS502 also labels subunit in immunocytochemistry applications. MS503 Complex V subunit mab 100 g of monoclonal antibody reactive against the F 1 F 0 ATP synthase (Complex V) (beta) subunit. MS503 also labels subunit in immunocytochemistry applications. MS504 Complex V subunit mab 100 g of monoclonal antibody reactive against the F 1 F 0 ATP synthase (Complex V) (delta) subunit. MS504 also labels the d subunit in immunocytochemistry applications. MS505 Complex V subunit OSCP mab 100 g of monoclonal antibody reactive against the F 1 F 0 ATP synthase (Complex V) OSCP subunit. MS505 also labels the OSCP subunit in immunocytochemistry applications. MS506 Complex V subunit IF 1 mab 100 g of monoclonal antibody reactive against complex V IF 1 subunit. The antibody shows in Western blotting of whole mitochondria shows reactivity with two bands at 10 kda and at 18 kda, but in isolated F 1 F 0 only the band at 10 kda is observed (IF 1 ). The 18 kda band is the subunit IV of Complex IV. MS508 Complex V subunit F 6 mab 100 g of monoclonal antibody reactive against the F 1 F 0 ATP synthase (Complex V) F 6 subunit. MS508 also labels the F 6 subunit in immunocytochemistry.

45 Alzheimer's Disease The involvement of mitochondria in Alzheimer's Disease has been known for many years with reduced cytochrome c oxidase assembly and activity considered as an early event in the disease, and reduced cytochrome c oxidase activity has been reported in platelets from patients with Alzheimer's Disease. Recent studies begin to explain mitochondrial involvement. The Alzheimer's amyloid precursor protein has now been localized to mitochondria as has the toxic amyloid beta peptide. The binding site for amyloid beta has been identified as alcohol dehydrogenase in the matrix space of the organelle. This enzyme can metabolize aldehydes and if the function is impaired could be involved in generation of oxidative radicals and a consequent cell death. Another mitochondrial protein implicated in Alzheimer's Disease is pyruvate dehydrogenase. Immunoassays MS401 MitoProfile Complex IV Immunocapture Kit 250 g, 500 g or 750 g monoclonal antibodies irreversibly crosslinked to protein G- agarose beads which can immunocapture ~25 g, ~50 g or ~75 g respectively of Complex IV from heart mitochondria. MS441 MitoProfile Rapid Microplate Assay Kit for Human Complex IV Activity A rapid activity microplate kit that is used to determine the activity of cytochrome c oxidase in a human sample with greater speed and simplicity. Includes buffers, detergent, substrates, and 96-well microplate with monoclonal antibody pre-bound to the wells of the plate, allowing for a stream-lined assay. MS442 MitoProfile Rapid Microplate Assay Kit for Human Complex IV Quantity A rapid activity microplate kit that is used to determine the amount of cytochrome c oxidase in a human sample with greater speed and simplicity. Includes buffers, detergent, substrates, and 96-well microplate with monoclonal antibody pre-bound to the wells of the plate, allowing for a stream-lined assay MS443 MitoProfile Rapid Microplate Assay Kit for Human Complex IV Activity and Quantity (Multiplexing) A 96-well multiplexing microplate kit that is used to determine both the activity and quantity of cytochrome c oxidase in a human sample with greater speed and simplicity. Includes buffers, detergent, substrates, and 96-well microplate with monoclonal antibody pre-bound to the wells of the plate, allowing for a stream-lined assay. MS444 MitoProfile Rapid Microplate Assay Kit for Mouse Complex IV Activity A 96-well activity microplate kit that is used to determine the activity of cytochrome c oxidase in a mouse sample with greater speed and simplicity. Includes buffers, detergent, substrates, and 96-well microplate with monoclonal antibody pre-bound to the wells of the plate, allowing for a stream-lined assay. 41

46 MS445 MitoProfile Rapid Microplate Assay Kit for Mouse Complex IV Quantity A 96-well activity microplate kit that is used to determine the amount of cytochrome c oxidase in a mouse sample with greater speed and simplicity. Includes buffers, detergent, substrates, and 96-well microplate with monoclonal antibody pre-bound to the wells of the plate, allowing for a stream-lined assay. MS446 MitoProfile Rapid Microplate Assay Kit for Mouse Complex IV Activity and Quantity (Multiplexing) A 96-well multiplexing microplate kit that is used to determine both the activity and quantity of cytochrome c oxidase in a mouse sample with greater speed and simplicity. Includes buffers, detergent, substrates, and 96-well microplate with monoclonal antibody pre-bound to the wells of the plate, allowing for a stream-lined assay. MS447 MitoProfile Rapid Microplate Assay Kit for Rat Complex IV Activity A 96-well activity microplate kit that is used to determine the activity of cytochrome c oxidase in a rat sample with greater speed and simplicity. Includes buffers, detergent, substrates, and 96-well microplate with monoclonal antibody pre-bound to the wells of the plate, allowing for a stream-lined assay. MS601 MitoProfile Human Total OXPHOS Complexes Detection Kit Contains a total of 360 g or 720 g of antibodies and is a premixed solution of 5 monoclonal antibodies against a subunit of each of the 5 OXPHOS complexes in mitochondrial preparations from human sources. Human heart mitochondria are also included as a Western blotting control. MS602 MitoProfile OXPHOS/PDH Immunocytochemistry Kit A cocktail of 220 g antibodies comprised of six monoclonal antibodies packaged individually. Each antibody is against a labile subunit of each of the 5 OXPHOS complexes and PDH. Each mab is provided in a 50 l aliquot at a 200x concentration, to allow reconstitution of each mab in 10 ml working volume. MS602a MitoProfile OXPHOS/PDH Immunocytochemistry Kit Includes the same components as MS602 (above), but additionally contains 60 g mab to porin, and 120 g to Complex V subunit, and the required secondary antibodies for simultaneous 2-dye staining of samples. MS604 MitoProfile Rodent Total OXPHOS Complexes Detection Kit Contains a total of 300 g or 600 g of antibodies and is a premixed solution of 5 monoclonal antibodies against a subunit of each of the 5 OXPHOS complexes in mitochondrial preparations from rodent sources. MSP01 MitoProfile PDH Immunocapture Kit 250 g monoclonal antibody irreversibly crosslinked to protein G-agarose beads which can immunocapture ~13 g of PDH complex from heart mitochondria. Dipstick Assays MS430 MitoProfile Dipstick Assay Kit for Human Complex IV Activity Contains 30 or 60 dipsticks and necessary components for quantitative analysis of cytochrome c oxidase activity in human and bovine samples.