Ubiquitin-like molecules (UBLs), such as SUMO1 (UBL1; MIM 601912), are structurally related to ubiquitin (MIM 191339) and can be ligated to target proteins in a similar manner as ubiquitin. However, covalent attachment of UBLs does not result in degradation of the modified proteins. SUMO1 modification is implicated in the targeting of RANGAP1 (MIM 602362) to the nuclear pore complex, as well as in stabilization of I-kappa-B-alpha (NFKBIA; MIM 112808) from degradation by the 26S proteasome. Like ubiquitin, UBLs are synthesized as precursor proteins, with 1 or more amino acids following the SLCterminal glycine-glycine residues of the mature UBL protein. Thus, the tail sequences of the UBL precursors need to be removed by UBL-specific proteases, such as SENP6, prior to their conjugation to target proteins (Kim et al., 2000 [PubMed 10799485]). SENPs also display isopeptidase activity for deconjugation of SUMO-conjugated substrates (Lima and Reverter, 2008 [PubMed 18799455]).[supplied by OMIM, Jun 2009]
Function:
Protease that deconjugates SUMO1, SUMO2 and SUMO3 from targeted proteins. Processes preferentially poly-SUMO2 and poly-SUMO3 chains, but does not efficiently process SUMO1, SUMO2 and SUMO3 precursors. Deconjugates SUMO1 from RXRA, leading to transcriptional activation. Involved in chromosome alignment and spindle assembly, by regulating the kinetochore CENPH-CENPI-CENPK complex. Desumoylates PML and CENPI, protecting them from degradation by the ubiquitin ligase RNF4, which targets polysumoylated proteins for proteasomal degradation. Desumoylates also RPA1, thus preventing recruitment of RAD51 to the DNA damage foci to initiate DNA repair through homologous recombinaison.
Subcellular Location:
Nucleus.
Tissue Specificity:
Highly expressed in reproductive organs, such as testis, ovary and prostate.
Similarity:
Belongs to the peptidase C48 family.
SWISS:
Q9GZR1
Gene ID:
26054
Database links:
Entrez Gene: 26054 Human
Entrez Gene: 421859 Chicken
Entrez Gene: 462829 Chimpanzee
Entrez Gene: 533853 Cow
Entrez Gene: 481883 Dog
Entrez Gene: 100068828 Horse
Entrez Gene: 215351 Mouse
Entrez Gene: 300860 Rat
Entrez Gene: 716554 Rhesus monkey
Omim: 605003 Human
SwissProt: Q9GZR1 Human
SwissProt: Q6P7W0 Mouse
Unigene: 485784 Human
Unigene: 28232 Mouse
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