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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) | |||
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) | |||
GFER | growth factor, augmenter of liver regeneration; Isoform 1- FAD-dependent sulfhydryl oxidase that regenerates the redox-active disulfide bonds in CHCHD4/MIA40, a chaperone essential for disulfide bond formation and protein folding in the mitochondrial intermembrane space. The reduced form of CHCHD4/MIA40 forms a transient intermolecular disulfide bridge with GFER/ERV1, resulting in regeneration of the essential disulfide bonds in CHCHD4/MIA40, while GFER/ERV1 becomes re- oxidized by donating electrons to cytochrome c or molecular oxygen (205 aa) | |||
MTX2 | metaxin 2; Involved in transport of proteins into the mitochondrion (263 aa) | |||
TOMM40 | translocase of outer mitochondrial membrane 40 homolog (yeast); Channel-forming protein essential for import of protein precursors into mitochondria (By similarity) (361 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) | |||
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) | |||
TOMM70A | translocase of outer mitochondrial membrane 70 homolog A (S. cerevisiae); Receptor that accelerates the import of all mitochondrial precursor proteins (By similarity) (608 aa) | |||
SFXN1 | sideroflexin 1; Might be involved in the transport of a component required for iron utilization into or out of the mitochondria (322 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) | |||
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) | |||
HSP90AA1 | heat shock protein 90kDa alpha (cytosolic), class A member 1; Molecular chaperone that promotes the maturation, structural maintenance and proper regulation of specific target proteins involved for instance in cell cycle control and signal transduction. Undergoes a functional cycle that is linked to its ATPase activity. This cycle probably induces conformational changes in the client proteins, thereby causing their activation. Interacts dynamically with various co-chaperones that modulate its substrate recognition, ATPase cycle and chaperone function (854 aa) | |||
UBC | ubiquitin C (685 aa) | |||
SAMM50 | sorting and assembly machinery component 50 homolog (S. cerevisiae); May be required for the assembly pathway of mitochondrial outer membrane proteins (By similarity) (469 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) | |||
PARK2 | parkinson protein 2, E3 ubiquitin protein ligase (parkin) (465 aa) | |||
DHX9 | DEAH (Asp-Glu-Ala-His) box polypeptide 9; Unwinds double-stranded DNA and RNA in a 3’ to 5’ direction. Alteration of secondary structure may subsequently influence interactions with proteins or other nucleic acids. Functions as a transcriptional activator. Component of the CRD- mediated complex that promotes MYC mRNA stability. Involved with LARP6 in the stabilization of type I collagen mRNAs for CO1A1 and CO1A2. Positively regulates HIV-1 LTR-directed gene expression (1270 aa) | |||
SFXN2 | sideroflexin 2; Potential iron transporter (322 aa) | |||
ABCC2 | ATP-binding cassette, sub-family C (CFTR/MRP), member 2; Mediates hepatobiliary excretion of numerous organic anions. May function as a cellular cisplatin transporter (1545 aa) | |||
HSPA1A | heat shock 70kDa protein 1A (641 aa) | |||
SLC25A6 | solute carrier family 25 (mitochondrial carrier; adenine nucleotide translocator), member 6; Catalyzes the exchange of cytoplasmic ADP with mitochondrial ATP across the mitochondrial inner membrane. May participate in the formation of the permeability transition pore complex (PTPC) responsible for the release of mitochondrial products that triggers apoptosis (298 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) | |||
TIMM9 | translocase of inner mitochondrial membrane 9 homolog (yeast); 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 (89 aa) | |||
TOMM6 | translocase of outer mitochondrial membrane 6 homolog (yeast) (74 aa) | |||
TOMM5 | translocase of outer mitochondrial membrane 5 homolog (yeast) (92 aa) |