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SLC25A3 | solute carrier family 25 (mitochondrial carrier; phosphate carrier), member 3; Transport of phosphate groups from the cytosol to the mitochondrial matrix. Phosphate is cotransported with H(+). May play a role regulation of the mitochondrial permeability transition pore (mPTP) (362 aa) | |||
EHHADH | enoyl-CoA, hydratase/3-hydroxyacyl CoA dehydrogenase (723 aa) | |||
PEPD | peptidase D (493 aa) | |||
TST | thiosulfate sulfurtransferase (rhodanese); Formation of iron-sulfur complexes, cyanide detoxification or modification of sulfur-containing enzymes. Other thiol compounds, besides cyanide, can act as sulfur ion acceptors. Also has weak mercaptopyruvate sulfurtransferase (MST) activity (By similarity). Together with MRPL18, acts as a mitochondrial import factor for the cytosolic 5S rRNA. Only the nascent unfolded cytoplasmic form is able to bind to the 5S rRNA (297 aa) | |||
CDY2A | chromodomain protein, Y-linked, 2A; May have histone acetyltransferase activity (By similarity) (541 aa) | |||
SQRDL | sulfide quinone reductase-like (yeast); Catalyzes the oxidation of hydrogen sulfide, with the help of a quinone (By similarity) (450 aa) | |||
NONO | non-POU domain containing, octamer-binding; DNA- and RNA binding protein, involved in several nuclear processes. Binds the conventional octamer sequence in double stranded DNA. Also binds single-stranded DNA and RNA at a site independent of the duplex site (By similarity). Involved in pre-mRNA splicing, probably as a heterodimer with SFPQ. Interacts with U5 snRNA, probably by binding to a purine-rich sequence located on the 3’ side of U5 snRNA stem 1b. The SFPQ-NONO heteromer associated with MATR3 may play a role in nuclear retention of defective RNAs. The SFPQ-NONO heteromer may be in [...] (471 aa) | |||
EXOG | endo/exonuclease (5’-3’), endonuclease G-like; Endo/exonuclease with nicking activity towards supercoiled DNA, a preference for single stranded DNA and 5’-3’ exonuclease activity (368 aa) | |||
ETHE1 | ethylmalonic encephalopathy 1; Probably plays an important role in metabolic homeostasis in mitochondria. May function as a nuclear-cytoplasmic shuttling protein that binds transcription factor RELA/NFKB3 in the nucleus and exports it to the cytoplasm. Suppresses p53- induced apoptosis by preventing nuclear localization of RELA (254 aa) | |||
CDYL2 | chromodomain protein, Y-like 2 (506 aa) | |||
ECI1 | enoyl-CoA delta isomerase 1; Able to isomerize both 3-cis and 3-trans double bonds into the 2-trans form in a range of enoyl-CoA species (302 aa) | |||
CDY1 | chromodomain protein, Y-linked, 1; Has histone acetyltransferase activity, with a preference for histone H4 (554 aa) | |||
CDY1B | chromodomain protein, Y-linked, 1B; Has histone acetyltransferase activity, with a preference for histone H4 (554 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) | |||
UCKL1 | uridine-cytidine kinase 1-like 1; May contribute to UTP accumulation needed for blast transformation and proliferation (548 aa) | |||
MOV10 | Mov10, Moloney leukemia virus 10, homolog (mouse); Probable RNA helicase. Required for RNA-mediated gene silencing by the RNA-induced silencing complex (RISC). Required for both miRNA-mediated translational repression and miRNA- mediated cleavage of complementary mRNAs by RISC. Also required for RNA-directed transcription and replication of the human hepatitis delta virus (HDV). Interacts with small capped HDV RNAs derived from genomic hairpin structures that mark the initiation sites of RNA-dependent HDV RNA transcription (1003 aa) | |||
ECHS1 | enoyl CoA hydratase, short chain, 1, mitochondrial; Straight-chain enoyl-CoA thioesters from C4 up to at least C16 are processed, although with decreasing catalytic rate (290 aa) | |||
XPNPEP2 | X-prolyl aminopeptidase (aminopeptidase P) 2, membrane-bound; A metalloprotease that may play a role in the inflammatory process and other reactions produced in response to injury or infection. May also play a role in the metabolism of the vasodilator bradykinin (674 aa) | |||
ENDOG | endonuclease G; Cleaves DNA at double-stranded (DG)n.(DC)n and at single-stranded (DC)n tracts. In addition to deoxyribonuclease activities, also has ribonuclease (RNase) and RNase H activities. Capable of generating the RNA primers required by DNA polymerase gamma to initiate replication of mitochondrial DNA (By similarity) (297 aa) | |||
AUH | AU RNA binding protein/enoyl-CoA hydratase; Catalyzes the conversion of 3-methylglutaconyl-CoA to 3- hydroxy-3-methylglutaryl-CoA. Has very low enoyl-CoA hydratase activity. Was originally identified as RNA-binding protein that binds in vitro to clustered 5’-AUUUA-3’ motifs (339 aa) | |||
ECI2 | enoyl-CoA delta isomerase 2 (394 aa) | |||
HADHA | hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit; Bifunctional subunit (763 aa) | |||
GLDC | glycine dehydrogenase (decarboxylating); The glycine cleavage system catalyzes the degradation of glycine. The P protein binds the alpha-amino group of glycine through its pyridoxal phosphate cofactor; CO(2) is released and the remaining methylamine moiety is then transferred to the lipoamide cofactor of the H protein (1020 aa) | |||
CDY2B | chromodomain protein, Y-linked, 2B; May have histone acetyltransferase activity (By similarity) (541 aa) | |||
HADH | hydroxyacyl-CoA dehydrogenase; Plays an essential role in the mitochondrial beta- oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA (331 aa) | |||
ECHDC1 | enoyl CoA hydratase domain containing 1 (307 aa) |