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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) | |||
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) | |||
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) | |||
SPG7 | spastic paraplegia 7 (pure and complicated autosomal recessive); Putative ATP-dependent zinc metalloprotease (795 aa) | |||
AFG3L2 | AFG3 ATPase family member 3-like 2 (S. cerevisiae); ATP-dependent protease which is essential for axonal development (By similarity) (797 aa) | |||
LETM1 | leucine zipper-EF-hand containing transmembrane protein 1; Crucial for the maintenance of mitochondrial tubular networks and for the assembly of the supercomplexes of the respiratory chain. Required for the maintenance of the tubular shape and cristae organization (739 aa) | |||
YME1L1 | YME1-like 1 (S. cerevisiae); Putative ATP-dependent protease which plays a role in mitochondrial protein metabolism. Ensures cell proliferation, maintains normal cristae morphology and complex I respiration activity, promotes antiapoptotic activity and protects mitochondria from the accumulation of oxidatively damaged membrane proteins. Requires to control the accumulation of nonassembled respiratory chain subunits (NDUFB6, OX4 and ND1). Seems to act in the processing of OPA1 (773 aa) | |||
TIMM22 | translocase of inner mitochondrial membrane 22 homolog (yeast); 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) (194 aa) | |||
ROMO1 | reactive oxygen species modulator 1; Induces production of reactive oxygen species (ROS) which are necessary for cell proliferation. May play a role in inducing oxidative DNA damage and replicative senescence. May play a role in the coordination of mitochondrial morphology and cell proliferation (79 aa) | |||
SGK2 | serum/glucocorticoid regulated kinase 2; Serine/threonine-protein kinase which is involved in the regulation of a wide variety of ion channels, membrane transporters, cell growth, survival and proliferation. Up- regulates Na(+) channels- SCNN1A/ENAC, K(+) channels- KCNA3/Kv1.3, KCNE1 and KCNQ1, amino acid transporter- SLC6A19, glutamate transporter- SLC1A6/EAAT4, glutamate receptors- GRIA1/GLUR1 and GRIK2/GLUR6, Na(+)/H(+) exchanger- SLC9A3/NHE3, and the Na(+)/K(+) ATPase (427 aa) | |||
TOX2 | TOX high mobility group box family member 2; Putative transcriptional activator involved in the hypothalamo-pituitary-gonadal system (506 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) | |||
OMA1 | OMA1 zinc metallopeptidase homolog (S. cerevisiae); Metalloprotease that is part of the quality control system in the inner membrane of mitochondria. Following stress conditions that induce loss of mitochondrial membrane potential, mediates cleavage of OPA1 at S1 position, leading to OPA1 inactivation and negative regulation of mitochondrial fusion. Its role in mitochondrial quality control is essential for regulating lipid metabolism as well as to maintain body temperature and energy expenditure under cold-stress conditions (524 aa) | |||
PMPCA | peptidase (mitochondrial processing) alpha; Cleaves presequences (transit peptides) from mitochondrial protein precursors (By similarity) (525 aa) | |||
ZMPSTE24 | zinc metallopeptidase STE24 homolog (S. cerevisiae); Proteolytically removes the C-terminal three residues of farnesylated proteins. Acts on lamin A/C (475 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) | |||
SDHD | succinate dehydrogenase complex, subunit D, integral membrane protein; Membrane-anchoring 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) (By similarity) (159 aa) | |||
TIMM17B | translocase of inner mitochondrial membrane 17 homolog B (yeast) (222 aa) | |||
IMMT | inner membrane protein, mitochondrial (758 aa) | |||
LETM2 | leucine zipper-EF-hand containing transmembrane protein 2 (444 aa) | |||
ENSG00000255292 | Uncharacterized protein (140 aa) |