UBE1
an enzyme of the ubiquitin-activating E1 family. Catalyzes the first step in ubiquitin conjugation to mark cellular proteins for degradation through the ubiquitin-proteasome system. Activates ubiquitin by first adenylating its C-terminal glycine residue with ATP, and thereafter linking this residue to the side chain of a cysteine residue in E1, yielding a ubiquitin-E1 thioester and free AMP. Essential for the formation of radiation-induced foci, timely DNA repair and for response to replication stress. Promotes the recruitment of TP53BP1 and BRCA1 at DNA damage sites. Belongs to the ubiquitin-activating E1 family. Detected in erythrocytes (at protein level). Ubiquitous. 2 alternative initiation human isoforms have been reported. Note: This description may include information from UniProtKB.
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Protein type: EC 6.3.2.19; Ubiquitin conjugating system; Ubiquitin ligase |
Chromosomal Location of Human Ortholog: Xp11.3 |
Cellular Component:
cytoplasm; cytosol; mitochondrion; nucleoplasm; nucleus
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Molecular Function:
ATP binding; protein binding; ubiquitin activating enzyme activity
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Biological Process:
cellular response to DNA damage stimulus; protein ubiquitination
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Disease: Spinal Muscular Atrophy, X-linked 2; Vexas Syndrome
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Reference #:
P22314
(UniProtKB)
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Alt. Names/Synonyms: A1S9; A1S9T; A1S9T and BN75 temperature sensitivity complementing; A1ST; AMCX1; CFAP124; GXP1; MGC4781; POC20; POC20 centriolar protein homolog; Protein A1S9; SMAX2; testicular secretory protein Li 63; UBA1; UBA1, ubiquitin-activating enzyme E1 homolog A; UBA1A; UBE1; UBE1X; ubiquitin like modifier activating enzyme 1; Ubiquitin-activating enzyme E1; ubiquitin-like modifier activating enzyme 1; Ubiquitin-like modifier-activating enzyme 1
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Gene Symbols: UBA1
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Molecular weight:
117,849 Da
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Basal Isoelectric point:
5.49
Predict pI for various phosphorylation states
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Protein-Specific Antibodies, siRNAs or Recombinant Proteins from Cell Signaling Technology®
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