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C1QBP | complement component 1, q subcomponent binding protein; Is believed to be a multifunctional and multicompartmental protein involved in inflammation and infection processes, ribosome biogenesis, regulation of apoptosis, transcriptional regulation and pre-mRNA splicing. At the cell surface is thought to act as an endothelial receptor for plasma proteins of the complement and kallikrein-kinin cascades. Putative receptor for C1q; specifically binds to the globular "heads" of C1q thus inhibiting C1; may perform the receptor function through a complex with C1qR/CD93. In complex with cytokera [...] (282 aa) | |||
ATP5E | ATP synthase, H+ transporting, mitochondrial F1 complex, epsilon subunit; 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 [...] (51 aa) | |||
PMPCB | peptidase (mitochondrial processing) beta; Cleaves presequences (transit peptides) from mitochondrial protein precursors (By similarity) (489 aa) | |||
COQ3 | coenzyme Q3 homolog, methyltransferase (S. cerevisiae) (369 aa) | |||
ERAL1 | Era G-protein-like 1 (E. coli); Probable GTPase that plays a role in the mitochondrial ribosomal small subunit assembly. Specifically binds the 12S mitochondrial rRNA (12S mt-rRNA) to a 33 nucleotide section delineating the 3’ terminal stem-loop region. May act as a chaperone that protects the 12S mt-rRNA on the 28S mitoribosomal subunit during ribosomal small subunit assembly (437 aa) | |||
COQ9 | coenzyme Q9 homolog (S. cerevisiae); Involved in the biosynthesis of coenzyme Q (By similarity) (318 aa) | |||
GOLPH3 | golgi phosphoprotein 3 (coat-protein); Mediates the cis and medial Golgi localization of mannosyltransferases through direct binding of their cytosolic domains. Involved in modulation of mTOR signaling. Involved in the regulation of mitochondrial lipids, leading to increase of mitochondrial mass. Potential oncogene (298 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) | |||
COQ5 | coenzyme Q5 homolog, methyltransferase (S. cerevisiae); Methyltransferase required for the conversion of 2- polyprenyl-6-methoxy-1,4-benzoquinol (DDMQH2) to 2-polyprenyl-3- methyl-6-methoxy-1,4-benzoquinol (DMQH2) (By similarity) (327 aa) | |||
ATP5O | ATP synthase, H+ transporting, mitochondrial F1 complex, O subunit; 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 [...] (213 aa) | |||
CLPB | ClpB caseinolytic peptidase B homolog (E. coli); May function as a regulatory ATPase and be related to secretion/protein trafficking process (707 aa) | |||
TMC1 | transmembrane channel-like 1; Probable ion channel required for the normal function of cochlear hair cells (By similarity) (760 aa) | |||
COQ4 | coenzyme Q4 homolog (S. cerevisiae); Component of the coenzyme Q biosynthetic pathway. May play a role in organizing a multi-subunit COQ enzyme complex required for coenzyme Q biosynthesis. Required for steady-state levels of other COQ polypeptides (265 aa) | |||
ATP5H | ATP synthase, H+ transporting, mitochondrial Fo complex, subunit d; 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 [...] (161 aa) | |||
COQ10A | coenzyme Q10 homolog A (S. cerevisiae); Required for the function of coenzyme Q in the respiratory chain. May serve as a chaperone or may be involved in the transport of Q6 from its site of synthesis to the catalytic sites of the respiratory complexes (Probable) (247 aa) | |||
ADCK4 | aarF domain containing kinase 4; The function of this protein is not yet clear. It is not known if it has protein kinase activity and what type of substrate it would phosphorylate (Ser, Thr or Tyr) (544 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) | |||
COQ7 | coenzyme Q7 homolog, ubiquinone (yeast); Involved in lifespan determination in ubiquinone- independent manner. Involved in ubiquinone biosynthesis. Potential central metabolic regulator (By similarity) (217 aa) | |||
COQ6 | coenzyme Q6 homolog, monooxygenase (S. cerevisiae) (468 aa) | |||
STOML2 | stomatin (EPB72)-like 2; Mitochondrial protein that probably regulates the biogenesis and the activity of mitochondria. Stimulates cardiolipin biosynthesis, binds cardiolipin-enriched membranes where it recruits and stabilizes some proteins including prohibitin and may therefore act in the organization of functional microdomains in mitochondrial membranes. Through regulation of the mitochondrial function may play a role into several biological processes including cell migration, cell proliferation, T-cell activation, calcium homeostasis and cellular response to stress. May play a role [...] (356 aa) | |||
ATP5C1 | ATP synthase, H+ transporting, mitochondrial F1 complex, gamma polypeptide 1; 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 cou [...] (298 aa) | |||
ADCK3 | aarF domain containing kinase 3; May be a chaperone-like protein essential for the proper conformation and functioning of protein complexes in the respiratory chain (647 aa) | |||
ATP5F1 | ATP synthase, H+ transporting, mitochondrial Fo complex, subunit B1; 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 [...] (256 aa) | |||
PMPCA | peptidase (mitochondrial processing) alpha; Cleaves presequences (transit peptides) from mitochondrial protein precursors (By similarity) (525 aa) | |||
PITRM1 | pitrilysin metallopeptidase 1 (1038 aa) | |||
CHCHD2 | coiled-coil-helix-coiled-coil-helix domain containing 2 (151 aa) |