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GOT2 | glutamic-oxaloacetic transaminase 2, mitochondrial (aspartate aminotransferase 2); Catalyzes the irreversible transamination of the L- tryptophan metabolite L-kynurenine to form kynurenic acid (KA). Plays a key role in amino acid metabolism. Important for metabolite exchange between mitochondria and cytosol. Facilitates cellular uptake of long-chain free fatty acids (430 aa) | |||
NDUFA2 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 2, 8kDa; Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (99 aa) | |||
NDUFA10 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 10, 42kDa; Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (355 aa) | |||
FAH | fumarylacetoacetate hydrolase (fumarylacetoacetase) (419 aa) | |||
SDHA | succinate dehydrogenase complex, subunit A, flavoprotein (Fp); Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). Can act as a tumor suppressor (664 aa) | |||
OXA1L | oxidase (cytochrome c) assembly 1-like; Required for the insertion of integral membrane proteins into the mitochondrial inner membrane. Essential for the activity and assembly of cytochrome oxidase. Required for the correct biogenesis of ATP synthase and complex I in mitochondria (495 aa) | |||
ATP1A1 | ATPase, Na+/K+ transporting, alpha 1 polypeptide; This is the catalytic component of the active enzyme, which catalyzes the hydrolysis of ATP coupled with the exchange of sodium and potassium ions across the plasma membrane. This action creates the electrochemical gradient of sodium and potassium ions, providing the energy for active transport of various nutrients (1023 aa) | |||
ATP1A3 | ATPase, Na+/K+ transporting, alpha 3 polypeptide; This is the catalytic component of the active enzyme, which catalyzes the hydrolysis of ATP coupled with the exchange of sodium and potassium ions across the plasma membrane. This action creates the electrochemical gradient of sodium and potassium ions, providing the energy for active transport of various nutrients (1013 aa) | |||
CYCS | cytochrome c, somatic; Electron carrier protein. The oxidized form of the cytochrome c heme group can accept an electron from the heme group of the cytochrome c1 subunit of cytochrome reductase. Cytochrome c then transfers this electron to the cytochrome oxidase complex, the final protein carrier in the mitochondrial electron-transport chain (By similarity) (105 aa) | |||
NDUFV2 | NADH dehydrogenase (ubiquinone) flavoprotein 2, 24kDa; Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity) (249 aa) | |||
NDUFA12 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 12; Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (145 aa) | |||
CS | citrate synthase (466 aa) | |||
GPT2 | glutamic pyruvate transaminase (alanine aminotransferase) 2; Catalyzes the reversible transamination between alanine and 2-oxoglutarate to form pyruvate and glutamate (523 aa) | |||
TOMM7 | translocase of outer mitochondrial membrane 7 homolog (yeast); Required for assembly and stability of the TOM complex (55 aa) | |||
SDHC | succinate dehydrogenase complex, subunit C, integral membrane protein, 15kDa; Membrane-anchoring subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q) (169 aa) | |||
GOT1 | glutamic-oxaloacetic transaminase 1, soluble (aspartate aminotransferase 1); Biosynthesis of L-glutamate from L-aspartate or L- cysteine. Important regulator of levels of glutamate, the major excitatory neurotransmitter of the vertebrate central nervous system. Acts as a scavenger of glutamate in brain neuroprotection. The aspartate aminotransferase activity is involved in hepatic glucose synthesis during development and in adipocyte glyceroneogenesis. Using L-cysteine as substrate, regulates levels of mercaptopyruvate, an important source of hydrogen sulfide. Mercaptopyruvate is conve [...] (413 aa) | |||
SDHB | succinate dehydrogenase complex, subunit B, iron sulfur (Ip); Iron-sulfur protein (IP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q) (280 aa) | |||
SDHD | succinate dehydrogenase complex, subunit D, integral membrane protein; Membrane-anchoring subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q) (By similarity) (159 aa) | |||
FXN | frataxin; Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe(2+) to proteins involved in these pathways. May play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe(2+) to Fe(3+); the oligomeric form but not the monomeric form has in vitro ferroxidase activity. May be able to store large amounts of iron in the form of a ferrihydrite mineral by oligomerization; however, the physiological relevance is unsure as reports are conflicting and the function has only been shown usin [...] (210 aa) | |||
FAHD1 | fumarylacetoacetate hydrolase domain containing 1; Probable mitochondrial acylpyruvase which is able to hydrolyze acetylpyruvate and fumarylpyruvate in vitro (248 aa) | |||
GPT | glutamic-pyruvate transaminase (alanine aminotransferase); Catalyzes the reversible transamination between alanine and 2-oxoglutarate to form pyruvate and glutamate. Participates in cellular nitrogen metabolism and also in liver gluconeogenesis starting with precursors transported from skeletal muscles (By similarity) (496 aa) | |||
ENSG00000249319 | Uncharacterized protein (293 aa) | |||
ACN9 | ACN9 homolog (S. cerevisiae) (125 aa) | |||
NDUFA6 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 6, 14kDa; Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed to be not involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (154 aa) | |||
SUCLG2 | succinate-CoA ligase, GDP-forming, beta subunit; Catalyzes the GTP-dependent ligation of succinate and CoA to form succinyl-CoA (By similarity) (440 aa) | |||
ENSG00000255292 | Uncharacterized protein (140 aa) |