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.
Protein type: EC; Ubiquitin conjugating system; Ubiquitin ligase
Chromosomal Location of Human Ortholog: Xp11.3
Cellular Component:  cytoplasm; cytosol; mitochondrion; nucleoplasm; nucleus
Molecular Function:  ATP binding; protein binding; ubiquitin activating enzyme activity
Biological Process:  cellular response to DNA damage stimulus; protein ubiquitination
Disease: Spinal Muscular Atrophy, X-linked 2; Vexas Syndrome
Reference #:  P22314 (UniProtKB)
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
Gene Symbols: UBA1
Molecular weight: 117,849 Da
Basal Isoelectric point: 5.49  Predict pI for various phosphorylation states
Protein-Specific Antibodies, siRNAs or Recombinant Proteins from Cell Signaling Technology® Total Proteins
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Protein Structure Not Found.

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