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| OTC | Ornithine carbamoyltransferase, mitochondrial; Ornithine carbamoyltransferase; Belongs to the ATCase/OTCase family (354 aa) | |||
| TIMM21 | Mitochondrial import inner membrane translocase subunit Tim21; Participates in the translocation of transit peptide- containing proteins across the mitochondrial inner membrane. Also required for assembly of mitochondrial respiratory chain complex I and complex IV as component of the MITRAC (mitochondrial translation regulation assembly intermediate of cytochrome c oxidase complex) complex. Probably shuttles between the presequence translocase and respiratory-chain assembly intermediates in a process that promotes incorporation of early nuclear-encoded subunits into these complexes; Be [...] (248 aa) | |||
| TIMM13 | Mitochondrial import inner membrane translocase subunit Tim13; Mitochondrial intermembrane chaperone that participates in the import and insertion of some multi-pass transmembrane proteins into the mitochondrial inner membrane. Also required for the transfer of beta-barrel precursors from the TOM complex to the sorting and assembly machinery (SAM complex) of the outer membrane. Acts as a chaperone-like protein that protects the hydrophobic precursors from aggregation and guide them through the mitochondrial intermembrane space. The TIMM8-TIMM13 complex mediates the import of proteins s [...] (95 aa) | |||
| TOMM22 | Mitochondrial import receptor subunit TOM22 homolog; Central receptor component of the translocase of the outer membrane of mitochondria (TOM complex) responsible for the recognition and translocation of cytosolically synthesized mitochondrial preproteins. Together with the peripheral receptor TOM20 functions as the transit peptide receptor and facilitates the movement of preproteins into the translocation pore; Belongs to the Tom22 family (142 aa) | |||
| ACO2 | Aconitate hydratase, mitochondrial; Catalyzes the isomerization of citrate to isocitrate via cis-aconitate; Belongs to the aconitase/IPM isomerase family (780 aa) | |||
| CMC2 | COX assembly mitochondrial protein 2 homolog; May be involved in cytochrome c oxidase biogenesis (79 aa) | |||
| TIMM10 | Mitochondrial import inner membrane translocase subunit Tim10; Mitochondrial intermembrane chaperone that participates in the import and insertion of multi-pass transmembrane proteins into the mitochondrial inner membrane. May also be required for the transfer of beta-barrel precursors from the TOM complex to the sorting and assembly machinery (SAM complex) of the outer membrane. Acts as a chaperone-like protein that protects the hydrophobic precursors from aggregation and guide them through the mitochondrial intermembrane space; Belongs to the small Tim family (90 aa) | |||
| COX17 | Cytochrome c oxidase copper chaperone; Copper chaperone for cytochrome c oxidase (COX). Binds two copper ions and deliver them to the Cu(A) site of COX (By similarity); Mitochondrial respiratory chain complex assembly factors (63 aa) | |||
| ATP5B | ATP synthase subunit beta, 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 c [...] (529 aa) | |||
| CHCHD3 | MICOS complex subunit MIC19; 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. Has also been shown to function as a transcription factor which binds to the BAG1 promoter and represses BAG1 transcription. Plays an important role in the maintenance of the MICOS complex stability and the mitochondrial cristae morphology; Mitochondrial coiled-coil-helix-coiled-coil-helix domain containing proteins (227 aa) | |||
| GRPEL1 | GrpE protein homolog 1, mitochondrial; 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 (By similarity). Seems to control the nucleotide-dependent binding of mitochondrial HSP70 to substrate proteins (217 aa) | |||
| VDAC1 | Voltage-dependent anion-selective channel protein 1; Forms a channel through the mitochondrial outer membrane and also the plasma membrane. The channel at the outer mitochondrial membrane allows diffusion of small hydrophilic molecules; in the plasma membrane it is involved in cell volume regulation and apoptosis. It adopts an open conformation at low or zero membrane potential and a closed conformation at potentials above 30-40 mV. The open state has a weak anion selectivity whereas the closed state is cation-selective. May participate in the formation of the permeability transition p [...] (283 aa) | |||
| TIMM44 | 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. Recruits mitochondrial HSP70 to drive protein translocation into the matrix using ATP as an energy source; Belongs to the Tim44 family (452 aa) | |||
| SLC25A4 | ADP/ATP translocase 1; Involved in mitochondrial ADP/ATP transport. Catalyzes the exchange of cytoplasmic ADP with mitochondrial ATP across the mitochondrial inner membrane; Belongs to the mitochondrial carrier (TC 2.A.29) family (298 aa) | |||
| TOMM70A | Mitochondrial import receptor subunit TOM70; Receptor that accelerates the import of all mitochondrial precursor proteins (608 aa) | |||
| NDUFB8 | NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial; 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 (186 aa) | |||
| LDHD | Probable D-lactate dehydrogenase, mitochondrial; Lactate dehydrogenase D; Belongs to the FAD-binding oxidoreductase/transferase type 4 family (507 aa) | |||
| CHCHD7 | Mitochondrial coiled-coil-helix-coiled-coil-helix domain containing proteins; Belongs to the CHCHD7 family (110 aa) | |||
| PAM16 | Mitochondrial import inner membrane translocase subunit TIM16; Regulates ATP-dependent protein translocation into the mitochondrial matrix. Inhibits DNAJC19 stimulation of HSPA9/Mortalin ATPase activity; Belongs to the TIM16/PAM16 family (125 aa) | |||
| TIMM22 | Mitochondrial import inner membrane translocase subunit Tim22; Essential core component of the TIM22 complex, a complex that mediates the import and insertion of multi-pass transmembrane proteins into the mitochondrial inner membrane. In the TIM22 complex, it constitutes the voltage-activated and signal-gated channel. Forms a twin-pore translocase that uses the membrane potential as external driving force in 2 voltage-dependent steps (By similarity); Belongs to the Tim17/Tim22/Tim23 family (194 aa) | |||
| CHCHD5 | Mitochondrial coiled-coil-helix-coiled-coil-helix domain containing proteins (110 aa) | |||
| GRPEL2 | GrpE protein homolog 2, mitochondrial; 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. Seems to control the nucleotide-dependent binding of mitochondrial HSP70 to substrate proteins. Stimulates ATPase activity of mt-HSP70. May also serve to modulate the interconversion of oligomeric (inactive) and monomeric (active) forms of mt-HSP70 (By similarity); Belongs to the GrpE family (225 aa) | |||
| COX19 | Cytochrome c oxidase assembly protein COX19; May be required for the assembly of mitochondrial cytochrome c oxidase; Mitochondrial respiratory chain complex assembly factors (90 aa) | |||
| COA4 | Cytochrome c oxidase assembly factor 4 homolog, mitochondrial; Putative COX assembly factor; Belongs to the COA4 family (87 aa) | |||
| TOMM7 | Mitochondrial import receptor subunit TOM7 homolog; Required for assembly and stability of the TOM complex. Positive regulator of PRKN translocation to damaged mitochondria. Acts probably by stabilizing PINK1 on the outer membrane of depolarized mitochondria; Belongs to the Tom7 family (55 aa) | |||
| TOMM20 | Mitochondrial import receptor subunit TOM20 homolog; Central component of the receptor complex responsible for the recognition and translocation of cytosolically synthesized mitochondrial preproteins. Together with TOM22 functions as the transit peptide receptor at the surface of the mitochondrion outer membrane and facilitates the movement of preproteins into the TOM40 translocation pore (By similarity); Belongs to the Tom20 family (145 aa) | |||
