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Symbol SUMO1 contributors: mct/ - updated : 29-09-2016
HGNC name SMT3 suppressor of mif two 3 homolog 1 (S. cerevisiae)
HGNC id 12502
interspecies homolog to yeast S.cerevisiae smt3 (see SMT3H1)
intraspecies homolog to SMT3H1
  • SMT3 subfamily
  • superfamily of ubiquitin-like proteins
  • SUMO subfamily
  • CATEGORY regulatory , transport
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,cytoskeleton,microtubule,mitotic spindle
    intracellular,nucleus,nucleoplasm,nuclear bodies,nuclear speckles
    intracellular,nuclear envelope,pore
  • localizes to the mitotic spindle and spindle midzone
  • basic FUNCTION
  • involved in posttranslational modifications of many proteins
  • protecting against both Fas/Apo-1 (TNFRSF6) or TNFR1 apoptosis, and antagonizing IKBA to generate protein resistant to degradation
  • involved in DNA repair topoisomerase mediated DNA damage
  • involved in NF-kappaB activation and may thus be involved in type 1 diabetes through apoptosis in pancreatic beta-cells
  • may control the activity of a repertoire of downstream effectors involved in palatogenesis
  • with other SUMO proteins, binding to lysine residues of target proteins and thereby modifying their stability, activity and subcellular localization
  • playing a role in meiotic chromosome synapsis
  • both SUMO1 and SUMO2 had the ability to enhance the solubility of MMP13, with the SUMO2 tag having a more significant effect
  • with SUMO2 and SUMO3 accumulate at DNA double-strand breaks sites in mammalian cells, with SUMO1 and SUMO2/3 accrual requiring the E3 ligase enzymes PIAS4 and PIAS1
  • covalently monoconjugated to proteins at lysine residues, thereby playing a critical role in many cellular processes, as mentioned earlier, through rapidly reversible changes in sumoylation status
  • plays an important role in modulation of NADPH oxidase complex (NOX) activity required for ROS generation
  • plays an essential role in ROS generation caused by NADPH oxidase activity
  • plays a key role in ROS generation by modifying NOX and inhibiting its enzymatic activity
  • is an important regulatory mechanism that indirectly represses the production of ROS to ameliorate cellular stress
  • CELLULAR PROCESS cell cycle, checkpoint
    cell life
    nucleotide, repair, recombination
    protein, degradation
    cell organization/biogenesis
    PHYSIOLOGICAL PROCESS immunity/defense , cellular trafficking transport
    text nucleo-cytoplasmic protein transport, stress response
  • SUMO pathway critically regulates PRDM1 function during plasma cell differentiation
  • a component
  • non-covalent ternary complex formed by PIAS1, SUMO1, and UBE2I proteins involved in transcriptional regulation
    small molecule
  • covalently linked to PML and NFKBIA via a conjugation step, requiring the activating enzymes SAE1 (AOS1) and SAE2 (UBA2)
  • associating with RAD51 and RAD52 proteins in the double strand break RAD51/RAD52 repair pathway
  • conjugating with TOP2A, TOP2B in response to DNA damage
  • conjugating to PCNA
  • interacting wiht DDXP1, TP53
  • targeting RANBP2
  • sumoylating ETV6 after conjugation of ETV6 by UBE2I
  • physically interacts with KLF4 in a region that matches an acidic and hydrophobic residue-rich SUMO-interacting motif (SIM) consensus
  • decreases JNK and MAPK14 activities, downstream of intracellular ROS signaling molecule, and increases cell viability response to heat-shock
  • partially sumoylates CYBB at plasma membrane and negatively modulates the ROS generation from CYBB
  • SUMOylation strongly enhances NKX2-5 transcriptional activity and that AA K51 of NKX2-5 is a SUMOylation target
  • ATP2A2 binds to SUMO1 and to ubiquitin-conjugating enzyme E2I (UBE2I)
  • more extensive contacts among SUMO, UBE2I, and RANBP2 in complexes containing SUMO1 compared with SUMO2
  • SUMO modification of the GLP1R could be a contributing factor to reduced incretin responsiveness
  • importance of SUMO modification of PCNA in preventing replication fork collapse to DNA double-strand breaks and providing genome stability
  • direct interaction between PGRMC1 and SUMO1, and this interaction is increased by progesterone
  • SUMO1, or more likely, a sumoylated protein, acts as an allosteric regulator of DPP9
  • DPP9 and SUMO1 interact in a SIM-independent manner, suggesting a novel surface on SUMO1 for noncovalent interactions with downstream effectors
  • noncovalent interaction of DPP9 with SUMO1 leads to activation of the peptidase
  • UBA2 interacts with both SUMO1 and SUMO2, both DPP8 and DPP9 showed a strong preference toward SUMO1 binding
  • CLOCK is a substrate of SUMO and sumoylation of CLOCK upregulates the transcriptional activity of ESR1
  • SUMO1 is involved in posttranslational modification of the lamin A tail
  • TRIM5 is a SUMO1 and SUMO2 substrate
  • AGO2 can be SUMOylated in mammalian cells by both SUMO1 and SUMO2
  • cell & other
    activated by E1 enzymes
    Other conjugating with TOP1
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional     --low  
    in autism with duplicated UBE3A
    haploinsufficiency leads to cleft lip and palate
    constitutional     --other  
    alterations in SUMO1 substrate conjugation may occur and global posttranslational modifications by ubiquitin may play an important role in the mechanisms underlying Alzheimer disease
    constitutional     --over  
    in isolated cardiomyocytes augmented contractility and accelerated Ca2+ decay
    Susceptibility to non-syndromic cleft lip with or without cleft palate (NSCLP)
    Variant & Polymorphism SNP significant correlations between a 4-SNP SUMO1 haplotype and NSCLP
    Candidate gene
    Therapy target
    cardiovascularaquiredheart failure
    targeting SUMO1 may provide a novel therapeutic strategy for the treatment of heart failure