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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) | |||
UQCRC1 | ubiquinol-cytochrome c reductase core protein I; 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 (480 aa) | |||
TOMM22 | translocase of outer mitochondrial membrane 22 homolog (yeast); 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 (142 aa) | |||
SFXN3 | sideroflexin 3; Potential iron transporter (325 aa) | |||
PMPCB | peptidase (mitochondrial processing) beta; Cleaves presequences (transit peptides) from mitochondrial protein precursors (By similarity) (489 aa) | |||
TIMM23 | translocase of inner mitochondrial membrane 23 homolog (yeast); Essential component of the TIM23 complex, a complex that mediates the translocation of transit peptide-containing proteins across the mitochondrial inner membrane (209 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) | |||
HSPA9 | heat shock 70kDa protein 9 (mortalin) (679 aa) | |||
PAM16 | presequence translocase-associated motor 16 homolog (S. cerevisiae); Regulates ATP-dependent protein translocation into the mitochondrial matrix. Inhibits DNAJC19 stimulation of HSPA9/Mortalin ATPase activity (125 aa) | |||
HAO2 | hydroxyacid oxidase 2 (long chain); Catalyzes the oxidation of L-alpha-hydroxy acids as well as, more slowly, that of L-alpha-amino acids (351 aa) | |||
SNRNP200 | small nuclear ribonucleoprotein 200kDa (U5); RNA helicase that plays an essential role in pre-mRNA splicing as component of the U5 snRNP and U4/U6-U5 tri-snRNP complexes. Involved in spliceosome assembly, activation and disassembly. Mediates changes in the dynamic network of RNA-RNA interactions in the spliceosome. Catalyzes the ATP-dependent unwinding of U4/U6 RNA duplices, an essential step in the assembly of a catalytically active spliceosome (2136 aa) | |||
TIMM50 | translocase of inner mitochondrial membrane 50 homolog (S. cerevisiae); Essential component of the TIM23 complex, a complex that mediates the translocation of transit peptide-containing proteins across the mitochondrial inner membrane. Has some phosphatase activity in vitro; however such activity may not be relevant in vivo. Isoform 2 may participate in the release of snRNPs and SMN from the Cajal body (456 aa) | |||
UBC | ubiquitin C (685 aa) | |||
TOMM7 | translocase of outer mitochondrial membrane 7 homolog (yeast); Required for assembly and stability of the TOM complex (55 aa) | |||
TOMM20 | translocase of outer mitochondrial membrane 20 homolog (yeast); 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) (145 aa) | |||
TIMM17A | translocase of inner mitochondrial membrane 17 homolog A (yeast); Essential component of the TIM23 complex, a complex that mediates the translocation of transit peptide-containing proteins across the mitochondrial inner membrane (171 aa) | |||
ASCC3 | activating signal cointegrator 1 complex subunit 3; 3’-5’ DNA helicase involved in repair of alkylated DNA. Promotes DNA unwinding to generate single-stranded substrate needed for ALKHB3, enabling ALKHB3 to process alkylated N3- methylcytosine (3mC) within double-stranded regions. Enhances NF- kappa-B, SRF and AP1 transactivation (2202 aa) | |||
SRSF3 | serine/arginine-rich splicing factor 3; May be involved in RNA processing in relation with cellular proliferation and/or maturation (164 aa) | |||
TIMM23B | translocase of inner mitochondrial membrane 23 homolog B (yeast); May participate in the translocation of transit peptide- containing proteins across the mitochondrial inner membrane. the PAM complex (By similarity) (171 aa) | |||
HAO1 | hydroxyacid oxidase (glycolate oxidase) 1; Has 2-hydroxyacid oxidase activity. Most active on the 2-carbon substrate glycolate, but is also active on 2-hydroxy fatty acids, with high activity towards 2-hydroxy palmitate and 2- hydroxy octanoate (370 aa) | |||
DNAJC15 | DnaJ (Hsp40) homolog, subfamily C, member 15 (150 aa) | |||
DNAJC19 | DnaJ (Hsp40) homolog, subfamily C, member 19; Probable 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. May act as a co-chaperone that stimulate the ATP-dependent activity (By similarity) (116 aa) | |||
TIMM17B | translocase of inner mitochondrial membrane 17 homolog B (yeast) (222 aa) | |||
TOMM6 | translocase of outer mitochondrial membrane 6 homolog (yeast) (74 aa) | |||
TOMM5 | translocase of outer mitochondrial membrane 5 homolog (yeast) (92 aa) | |||
CRN7 | CORO7-PAM16 readthrough; May play a role in the maintenance of the Golgi apparatus morphology and in the protein export from the Golgi (1048 aa) |