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TIMM21 | translocase of inner mitochondrial membrane 21 homolog (yeast); 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 some MITRAC complex, a cytochrome c oxidase (COX) assembly intermediate complex. TIM21 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 (248 aa) | |||
COX7C | cytochrome c oxidase subunit VIIc; This protein is one of the nuclear-coded polypeptide chains of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport (63 aa) | |||
PMPCB | peptidase (mitochondrial processing) beta; Cleaves presequences (transit peptides) from mitochondrial protein precursors (By similarity) (489 aa) | |||
COX5B | cytochrome c oxidase subunit Vb; This protein is one of the nuclear-coded polypeptide chains of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport (129 aa) | |||
GRPEL1 | GrpE-like 1, mitochondrial (E. coli); 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 (217 aa) | |||
UQCRC2 | ubiquinol-cytochrome c reductase core protein II; This is a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain. The core protein 2 is required for the assembly of the complex (453 aa) | |||
NUDT7 | nudix (nucleoside diphosphate linked moiety X)-type motif 7; Coenzyme A diphosphatase which mediates the cleavage of CoA, CoA esters and oxidized CoA with similar efficiencies, yielding 3’,5’-ADP and the corresponding 4’-phosphopantetheine derivative as products. CoA into 3’,5’-ADP and 4’- phosphopantetheine. Has no activity toward NDP-sugars, CDP- alcohols, (deoxy)nucleoside 5’-triphosphates, nucleoside 5’-di or monophosphates, diadenosine polyphosphates, NAD, NADH, NADP, NADPH or thymidine-5’-monophospho-p-nitrophenyl ester. May be required to eliminate oxidized CoA from peroxisomes, [...] (238 aa) | |||
TIMM44 | translocase of inner mitochondrial membrane 44 homolog (yeast); 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 (452 aa) | |||
UQCRB | ubiquinol-cytochrome c reductase binding protein; This is a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain. This component is involved in redox-linked proton pumping (111 aa) | |||
HSPA9 | heat shock 70kDa protein 9 (mortalin) (679 aa) | |||
NUDT8 | nudix (nucleoside diphosphate linked moiety X)-type motif 8; Probably mediates the hydrolysis of some nucleoside diphosphate derivatives (By similarity) (140 aa) | |||
UQCRFS1 | ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1; Component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is a respiratory chain that generates an electrochemical potential coupled to ATP synthesis (274 aa) | |||
UQCRH | ubiquinol-cytochrome c reductase hinge protein; This is a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain. This protein may mediate formation of the complex between cytochromes c and c1 (91 aa) | |||
CYC1 | cytochrome c-1; This is the heme-containing component of the cytochrome b-c1 complex, which accepts electrons from Rieske protein and transfers electrons to cytochrome c in the mitochondrial respiratory chain (325 aa) | |||
COX5A | cytochrome c oxidase subunit Va; This is the heme A-containing chain of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport (150 aa) | |||
GRPEL2 | GrpE-like 2, mitochondrial (E. coli); 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) (225 aa) | |||
HN1 | hematological and neurological expressed 1 (181 aa) | |||
WTAP | Wilms tumor 1 associated protein; Regulates G2/M cell-cycle transition by binding to the 3’ UTR of CCNA2, which enhances its stability. Impairs WT1 DNA- binding ability and inhibits expression of WT1 target genes. May be involved in mRNA splicing regulation (396 aa) | |||
MT-CO1 | mitochondrially encoded cytochrome c oxidase I; 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 (By similarity) (513 aa) | |||
MT-CYB | mitochondrially encoded cytochrome b; Component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is a respiratory chain that generates an electrochemical potential coupled to ATP synthesis (By similarity) (380 aa) | |||
MT-CO2 | mitochondrially encoded cytochrome c oxidase II; 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. Subunit 2 transfers the electrons from cytochrome c via its binuclear copper A center to the bimetallic center of the catalytic subunit 1 (By similarity) (227 aa) | |||
PMPCA | peptidase (mitochondrial processing) alpha; Cleaves presequences (transit peptides) from mitochondrial protein precursors (By similarity) (525 aa) | |||
EXOSC2 | exosome component 2; Non-catalytic component of the RNA exosome complex which has 3’->5’ exoribonuclease activity and participates in a multitude of cellular RNA processing and degradation events. In the nucleus, the RNA exosome complex is involved in proper maturation of stable RNA species such as rRNA, snRNA and snoRNA, in the elimination of RNA processing by-products and non-coding ’pervasive’ transcripts, such as antisense RNA species and promoter-upstream transcripts (PROMPTs), and of mRNAs with processing defects, thereby limiting or excluding their export to the cytoplasm. The R [...] (293 aa) | |||
RPA2 | replication protein A2, 32kDa; Required for DNA recombination, repair and replication. The activity of RP-A is mediated by single-stranded DNA binding and protein interactions. Required for the efficient recruitment of the DNA double-strand break repair factor RAD51 to chromatin in response to DNA damage (270 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) | |||
UQCRQ | ubiquinol-cytochrome c reductase, complex III subunit VII, 9.5kDa; This is a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain. This subunit, together with cytochrome b, binds to ubiquinone (82 aa) |