CARTMitochondrial Proteins
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Aconitase
Aconitase is an iron-sulfur protein that catalyzes the reversible interconversion of citrate and isocitrate, via a cis-aconitate intermediate, in both the TCA and glyoxylate cycles. |
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Apoptosis-Inducing Factor (AIF)
AIF is a flavoprotein localized in the inter-membrane space. It is a multi-functional protein with a vital oxidoreductase function, an anti-oxidant activity, an assembly function in Complex I formation, and an apoptogenic function. |
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Adenine Nucleotide Translocase (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. |
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ATP synthase
ATP synthase is responsible for ATP production in oxidative phosphorylation and can work in reverse as a proton pumping ATPase. |
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Complex I
Complex I catalyses electron entry from NADH via a flavin (FMN) and several non-heme iron centers. |
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Complex II
Complex II is both a component of the electron transport chain and an enzyme of the Krebs cycle. |
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Complex III
Complex III catalyses electron transfer from reduced ubiquinone or coenzyme Q 10 to cytochrome c. |
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Complex IV
Complex IV is the last enzyme in the respiratory electron transport chain and it helps to establish a transmembrane difference of proton electrochemical potential that the ATP synthase then uses to synthesize ATP. |
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Cyclophilin D
Cyclophilin D is the binding site for cyclosporins and this protein may be the gate for PTP. |
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Cytochrome c
Cytochrome c plays crucial roles in both oxidative phosphorylation and in apoptosis. |
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L-xylulose reductase (DCXR)
DCXR is an oxidoreductase enzyme that catalyzes the NADH-dependent reduction of several pentoses, tetroses, alpha-dicarbonyl compounds and L-xylulose. |
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Frataxin
The specific function of frataxin is still unknown, but it has been shown to play a role in iron metabolism. |
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Medium-Chain Acyl CoA Dehydrogenase (MCAD)
MCAD is an oxidoreductase enzyme of the mitochondrial fatty acid beta-oxidation pathway that is specific for acyl chain lengths of 4 to 16. |
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Mitofilin
Mitofilin has critical functions in mitochondrial morphology and mitochondrial fusion and fission, specifically in the formation of tubular cristae and cristae junctions. |
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Nicotinamide nucleotide transhydrogenase (NNT)
NNT is a mitochondrial inner membrane protein catalyzing the interconversion of NADH and NADPH within the matrix space of the organelle. |
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Pyruvate dehydrogenase (PDH)
The pyruvate dehydrogenase complex (PDH) is at the centre of aerobic carbohydrate metabolism. |
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Porin
The channel-forming protein porin (also called the voltage dependent anion channel, VDAC) is involved in both aerobic metabolism and apoptotic processes. |
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Short-Chain 3-Hydroxyacyl-CoA Dehydrogenase (SCHAD)
Plays an essential role in the mitochondrial beta-oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA. |
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Mitochondrial Superoxide Dismutase 2 (SOD2)
The principle cellular anti-oxidants are the superoxide dismutase family (SOD). |
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Mitochondrial Trifunctional Protein (TFP)
TFP 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. |
