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FLASH GENE
Symbol UBA1 contributors: mct/npt/shn - updated : 21-03-2018
HGNC name ubiquitin-like modifier activating enzyme 1
HGNC id 12469
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • C-terminal ubiquitin-fold domain
  • mono polymer monomer
    HOMOLOGY
    interspecies ortholog to Uba1, Mus musculus
    ortholog to Uba1, Rattus norvegicus
    ortholog to uba1, Danio rerio
    ortholog to UBA1, Pan troglodytes
    Homologene
    FAMILY
  • ubiquitin-activating E1 family
  • CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     plasma membrane
        intracellular
    intracellular,cytoplasm,cytosolic
    text
  • reduced UBA1 localizes mainly in the cytosol, but is translocated from the cytosol to the membrane under conditions of oxidative stress
  • basic FUNCTION
  • ubiquitin-activating enzyme E1, escaping inactivation in human involved in cell cycle progression through S phase
  • catalyzing the first step in ubiquitin conjugation to mark cellular proteins for degradation
  • required for charging the cell-cycle E2s Cdc34A and Cdc34B (
  • major function of UBA1 is to promote degradation of damaged proteins by 26S proteasome by tagging the target proteins with an Ub moiety
  • ubiquitination levels are strongly related to UBA1 activity
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • component of a complex with UBE2E2, EBE2D1, UBE2G1, CBL, UBE3
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • Hsp105alpha (
  • interactions between FUS and proteins involved in neurodegenerative diseases and/or ubiquitin proteasome pathway, such as VCP, SFPQ, UBA1, and 26S proteosome non-ATPase regulatory subunit 12 (PSMD12)
  • UBA1 and UBD form a stable non-reducible conjugate under overexpression as well as under endogenous conditions after induction of endogenous UBD expression with proinflammatory cytokines
  • does not act as a second E1 activating enzyme for UBD but UBDylation of UBA1 leads to its proteasomal degradation, implying a putative regulatory role of UBD in the ubiquitin conjugation pathway
  • cell & other
    REGULATION
    Phosphorylated by protein kinase Cdc2 (
    Other UBA1 Cys-278 is sensitive to oxidation and can affect UB charging through a change in its proximity to UB
    ASSOCIATED DISORDERS
    corresponding disease(s) SMAX
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS