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GALK1 | galactokinase 1; Major enzyme for galactose metabolism (392 aa) | |||
PUS3 | pseudouridylate synthase 3; Formation of pseudouridine at position 39 in the anticodon stem and loop of transfer RNAs (By similarity) (481 aa) | |||
MOCS3 | molybdenum cofactor synthesis 3; Plays a central role in 2-thiolation of mcm(5)S(2)U at tRNA wobble positions of tRNA(Lys), tRNA(Glu) and tRNA(Gln). Also essential during biosynthesis of the molybdenum cofactor. Acts by mediating the C-terminal thiocarboxylation of sulfur carriers URM1 and MOCS2A. Its N-terminus first activates URM1 and MOCS2A as acyl-adenylates (-COAMP), then the persulfide sulfur on the catalytic cysteine is transferred to URM1 and MOCS2A to form thiocarboxylation (-COSH) of their C-terminus. The reaction probably involves hydrogen sulfide that is generated from the [...] (460 aa) | |||
SSB | Sjogren syndrome antigen B (autoantigen La); Binds to the 3’ poly(U) terminii of nascent RNA polymerase III transcripts, protecting them from exonuclease digestion and facilitating their folding and maturation (408 aa) | |||
HUWE1 | HECT, UBA and WWE domain containing 1, E3 ubiquitin protein ligase (4374 aa) | |||
TRMT10A | tRNA methyltransferase 10 homolog A (S. cerevisiae); Probable RNA methyltransferase (By similarity) (339 aa) | |||
PSPH | phosphoserine phosphatase; Catalyzes the last step in the biosynthesis of serine from carbohydrates. The reaction mechanism proceeds via the formation of a phosphoryl-enzyme intermediates (225 aa) | |||
NOL6 | nucleolar protein family 6 (RNA-associated) (1146 aa) | |||
TRMT10B | tRNA methyltransferase 10 homolog B (S. cerevisiae); Probable RNA methyltransferase (By similarity) (316 aa) | |||
TRUB1 | TruB pseudouridine (psi) synthase homolog 1 (E. coli); May be responsible for synthesis of pseudouridine from uracil in transfer RNAs (By similarity) (349 aa) | |||
TRMT10C | tRNA methyltransferase 10 homolog C (S. cerevisiae); Functions in mitochondrial tRNA maturation. Part of mitochondrial ribonuclease P, an enzyme composed of MRPP1/RG9MTD1, MRPP2/HSD17B10 and MRPP3/KIAA0391, which cleaves tRNA molecules in their 5’-ends (403 aa) | |||
LARP7 | La ribonucleoprotein domain family, member 7; Negative transcriptional regulator of polymerase II genes, acting by means of the 7SK RNP system. Within the 7SK RNP complex, the positive transcription elongation factor b (P-TEFb) is sequestered in an inactive form, preventing RNA polymerase II phosphorylation and subsequent transcriptional elongation (582 aa) | |||
METTL1 | methyltransferase like 1; Catalyzes the formation of N(7)-methylguanine at position 46 (m7G46) in tRNA (276 aa) | |||
MAF1 | MAF1 homolog (S. cerevisiae); Element of the mTORC1 signaling pathway that acts as a mediator of diverse signals and that represses RNA polymerase III transcription. Inhibits the de novo assembly of TFIIIB onto DNA (256 aa) | |||
XPOT | exportin, tRNA; Mediates the nuclear export of aminoacylated tRNAs. In the nucleus binds to tRNA and to the GTPase Ran in its active GTP- bound form. Docking of this trimeric complex to the nuclear pore complex (NPC) is mediated through binding to nucleoporins. Upon transit of a nuclear export complex into the cytoplasm, disassembling of the complex and hydrolysis of Ran-GTP to Ran-GDP (induced by RANBP1 and RANGAP1, respectively) cause release of the tRNA from the export receptor. XPOT then return to the nuclear compartment and mediate another round of transport. The directionality of [...] (962 aa) | |||
WDR4 | WD repeat domain 4; Required for the formation of N(7)-methylguanine at position 46 (m7G46) in tRNA. In the complex, it is required to stabilize and induce conformational change of the catalytic subunit (412 aa) | |||
MIOS | missing oocyte, meiosis regulator, homolog (Drosophila) (875 aa) | |||
AP3D1 | adaptor-related protein complex 3, delta 1 subunit; Part of the AP-3 complex, an adaptor-related complex which is not clathrin-associated. The complex is associated with the Golgi region as well as more peripheral structures. It facilitates the budding of vesicles from the Golgi membrane and may be directly involved in trafficking to lysosomes. In concert with the BLOC-1 complex, AP-3 is required to target cargos into vesicles assembled at cell bodies for delivery into neurites and nerve terminals (1153 aa) | |||
UBC | ubiquitin C (685 aa) | |||
WWP2 | WW domain containing E3 ubiquitin protein ligase 2 (870 aa) | |||
SMURF1 | SMAD specific E3 ubiquitin protein ligase 1; E3 ubiquitin-protein ligase that acts as a negative regulator of BMP signaling pathway. Mediates ubiquitination and degradation of SMAD1 and SMAD5, 2 receptor-regulated SMADs specific for the BMP pathway. Promotes ubiquitination and subsequent proteasomal degradation of TRAF family members and RHOA (757 aa) | |||
DKC1 | dyskeratosis congenita 1, dyskerin; Isoform 1- Required for ribosome biogenesis and telomere maintenance. Probable catalytic subunit of H/ACA small nucleolar ribonucleoprotein (H/ACA snoRNP) complex, which catalyzes pseudouridylation of rRNA. This involves the isomerization of uridine such that the ribose is subsequently attached to C5, instead of the normal N1. Each rRNA can contain up to 100 pseudouridine (’psi’) residues, which may serve to stabilize the conformation of rRNAs. Also required for correct processing or intranuclear trafficking of TERC, the RNA component of the telomera [...] (514 aa) | |||
ITCH | itchy E3 ubiquitin protein ligase (862 aa) | |||
URM1 | ubiquitin related modifier 1; Acts as a sulfur carrier required for 2-thiolation of mcm(5)S(2)U at tRNA wobble positions of tRNA(Lys), tRNA(Glu) and tRNA(Gln). Serves as sulfur donor in tRNA 2-thiolation reaction by thiocarboxylated (-COSH) at its C-terminus by MOCS3. The sulfur is then transferred to tRNA to form 2-thiolation of mcm(5)S(2)U. May also act as an ubiquitin-like protein that is covalently conjugated to other proteins; the relevance of such function is however unclear in vivo (146 aa) | |||
GALK2 | galactokinase 2; Acts on GalNAc. Also acts as a galactokinase when galactose is present at high concentrations. May be involved in a salvage pathway for the reutilization of free GalNAc derived from the degradation of complex carbohydrates (458 aa) |