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A0A1L9SD61 | Magnesium transporter. (399 aa) | ||||
GUF1 | Translation factor GUF1, mitochondrial; Promotes mitochondrial protein synthesis. May act as a fidelity factor of the translation reaction, by catalyzing a one-codon backward translocation of tRNAs on improperly translocated ribosomes. Binds to mitochondrial ribosomes in a GTP-dependent manner. (664 aa) | ||||
COQ3 | Ubiquinone biosynthesis O-methyltransferase, mitochondrial; O-methyltransferase that catalyzes the 2 O-methylation steps in the ubiquinone biosynthetic pathway; Belongs to the class I-like SAM-binding methyltransferase superfamily. UbiG/COQ3 family. (349 aa) | ||||
A0A1L9S9M6 | ATP synthase subunit d, mitochondrial; Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase or Complex V) produces ATP from ADP in the presence of a proton gradient across the membrane which is generated by electron transport complexes of the respiratory chain. F-type ATPases consist of two structural domains, F(1) - containing the extramembraneous catalytic core, and F(0) - containing the membrane proton channel, linked together by a central stalk and a peripheral stalk. During catalysis, ATP synthesis in the catalytic domain of F(1) is coupled via a rotary mechanism of the cent [...] (169 aa) | ||||
COQ4 | Ubiquinone biosynthesis protein COQ4, mitochondrial; Component of the coenzyme Q biosynthetic pathway. May play a role in organizing a multi-subunit COQ enzyme complex required for coenzyme Q biosynthesis. Required for steady-state levels of other COQ polypeptides; Belongs to the COQ4 family. (285 aa) | ||||
A0A1L9S8E0 | SURF1-like protein; Probably involved in the biogenesis of the COX complex. Belongs to the SURF1 family. (322 aa) | ||||
A0A1L9S8C3 | Cytochrome c oxidase subunit 6A, mitochondrial. (133 aa) | ||||
A0A1L9S7E4 | Magnesium transporter. (585 aa) | ||||
COQ6 | Ubiquinone biosynthesis monooxygenase COQ6, mitochondrial; FAD-dependent monooxygenase required for the C5-ring hydroxylation during ubiquinone biosynthesis. Catalyzes the hydroxylation of 3-polyprenyl-4-hydroxybenzoic acid to 3-polyprenyl- 4,5-dihydroxybenzoic acid. The electrons required for the hydroxylation reaction may be funneled indirectly from NADPH via a ferredoxin/ferredoxin reductase system to COQ6. (512 aa) | ||||
A0A1L9SPX1 | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit; 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. (189 aa) | ||||
A0A1L9SPR9 | NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial; 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. (498 aa) | ||||
A0A1L9SNN2 | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit; 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. (142 aa) | ||||
COQ7 | 5-demethoxyubiquinone hydroxylase, mitochondrial; Catalyzes the hydroxylation of 2-polyprenyl-3-methyl-6- methoxy-1,4-benzoquinol (DMQH2) during ubiquinone biosynthesis. Has also a structural role in the COQ enzyme complex, stabilizing other COQ polypeptides. (237 aa) | ||||
A0A1L9S7A9 | Mitochondrial pyruvate carrier; Mediates the uptake of pyruvate into mitochondria. Belongs to the mitochondrial pyruvate carrier (MPC) (TC 2.A.105) family. (179 aa) | ||||
A0A1L9S4A3 | Cytochrome c oxidase subunit 1; Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Subunits 1-3 form the functional core of the enzyme complex. CO I is the catalytic subunit of the enzyme. Electrons originating in cytochrome c are transferred via the copper A center of subunit 2 and heme A of subunit 1 to the bimetallic center formed by heme A3 and copper B. (127 aa) | ||||
A0A1L9SC48 | Cytochrome c heme lyase; Links covalently the heme group to the apoprotein of cytochrome c. (290 aa) | ||||
A0A1L9SLD5 | COX assembly mitochondrial protein; Required for mitochondrial cytochrome c oxidase (COX) assembly and respiration; Belongs to the CMC family. (62 aa) | ||||
A0A1L9SGT7 | Cytochrome c heme lyase; Links covalently the heme group to the apoprotein of cytochrome c. (571 aa) | ||||
A0A1L9SCD0 | Cytochrome b-c1 complex subunit 7; Component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain; Belongs to the UQCRB/QCR7 family. (104 aa) | ||||
A0A1L9SF67 | Succinate dehydrogenase [ubiquinone] cytochrome b small subunit. (164 aa) | ||||
A0A1L9SW53 | Cytochrome c oxidase assembly protein COX20, mitochondrial; Involved in the assembly of the cytochrome c oxidase complex. Belongs to the COX20 family. (203 aa) | ||||
A0A1L9SC08 | MICOS complex subunit MIC60; Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane. (1904 aa) | ||||
A0A1L9SBY3 | Mitochondrial pyruvate carrier; Mediates the uptake of pyruvate into mitochondria. Belongs to the mitochondrial pyruvate carrier (MPC) (TC 2.A.105) family. (103 aa) | ||||
PSD1 | Phosphatidylserine decarboxylase proenzyme 1, mitochondrial; Catalyzes the formation of phosphatidylethanolamine (PtdEtn) from phosphatidylserine (PtdSer). Plays a central role in phospholipid metabolism and in the interorganelle trafficking of phosphatidylserine. Belongs to the phosphatidylserine decarboxylase family. PSD-B subfamily. Eukaryotic type I sub-subfamily. (546 aa) | ||||
A0A1L9SEQ3 | COX assembly mitochondrial protein; Required for mitochondrial cytochrome c oxidase (COX) assembly and respiration; Belongs to the CMC family. (124 aa) | ||||
A0A1L9SE36 | MICOS complex subunit; Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane. (251 aa) | ||||
A0A1L9SUM9 | Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial; 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). (274 aa) | ||||
A0A1L9SUJ1 | Sensitive to high expression protein 9, mitochondrial; Required for the maintenance of the structure of the mitochondrial inner membrane. Involved in mitochondrial morphology. (509 aa) | ||||
A0A1L9STX9 | Prohibitin. (305 aa) | ||||
A0A1L9STK9 | Mitochondrial inner membrane protease ATP23; Has a dual role in the assembly of mitochondrial ATPase. Belongs to the peptidase M76 family. (239 aa) | ||||
A0A1L9SS01 | NADH-ubiquinone oxidoreductase; 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. (159 aa) | ||||
A0A1L9SRX3 | Prohibitin. (277 aa) | ||||
A0A1L9SRU8 | MICOS complex subunit MIC10; Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane. (91 aa) | ||||
A0A1L9SR95 | Mitochondrial import inner membrane translocase subunit TIM44; Essential component of the PAM complex, a complex required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner; Belongs to the Tim44 family. (513 aa) | ||||
A0A1L9SQY2 | 4-hydroxybenzoate polyprenyltransferase, mitochondrial; Catalyzes the prenylation of para-hydroxybenzoate (PHB) with an all-trans polyprenyl group. Mediates the second step in the final reaction sequence of coenzyme Q (CoQ) biosynthesis, which is the condensation of the polyisoprenoid side chain with PHB, generating the first membrane-bound Q intermediate. (420 aa) | ||||
A0A1L9SQT8 | MICOS complex subunit MIC12; Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane. (125 aa) | ||||
A0A1L9SQK1 | Mitochondrial glycine transporter; Mitochondrial glycine transporter that imports glycine into the mitochondrial matrix. Plays an important role in providing glycine for the first enzymatic step in heme biosynthesis, the condensation of glycine with succinyl-CoA to produce 5-aminolevulinate (ALA) in the miochondrial matrix; Belongs to the mitochondrial carrier (TC 2.A.29) family. SLC25A38 subfamily. (330 aa) | ||||
COQ5 | 2-methoxy-6-polyprenyl-1,4-benzoquinol methylase, mitochondrial; Methyltransferase required for the conversion of 2- polyprenyl-6-methoxy-1,4-benzoquinol (DDMQH2) to 2-polyprenyl-3-methyl- 6-methoxy-1,4-benzoquinol (DMQH2). (311 aa) |