ID:UBP1_HUMAN DESCRIPTION: RecName: Full=Ubiquitin carboxyl-terminal hydrolase 1; EC=3.4.19.12; AltName: Full=Deubiquitinating enzyme 1; Short=hUBP; AltName: Full=Ubiquitin thioesterase 1; AltName: Full=Ubiquitin-specific-processing protease 1; FUNCTION: Negative regulator of DNA damage repair which specifically deubiquitinates monoubiquitinated FANCD2. Also involved in PCNA-mediated translesion synthesis (TLS) by deubiquitinating monoubiquitinated PCNA. Has almost no deubiquitinating activity by itself and requires the interaction with WDR48 to have a high activity. CATALYTIC ACTIVITY: Thiol-dependent hydrolysis of ester, thioester, amide, peptide and isopeptide bonds formed by the C- terminal Gly of ubiquitin (a 76-residue protein attached to proteins as an intracellular targeting signal). BIOPHYSICOCHEMICAL PROPERTIES: Kinetic parameters: KM=0.7 uM for ubiquitin vinyl sulfone (in presence of WDR48); KM=1.4 uM for ubiquitin vinyl sulfone (in absence of WDR48); SUBUNIT: Interacts with FANCD2 and PCNA. Interacts with WDR48. SUBCELLULAR LOCATION: Nucleus. DEVELOPMENTAL STAGE: Cell cycle-regulated. Highest level during S phase. INDUCTION: Down-regulated following DNA damage. PTM: Autocatalytic cleavage of USP1 following UV irradiation inactivates it leading to an increase in ubiquitinated PCNA, recruitment of POLH and translesion synthesis. PTM: Ubiquitinated; leading to its subsequent proteasomal degradation (By similarity). PTM: Phosphorylated upon DNA damage, probably by ATM or ATR. MISCELLANEOUS: HEK293T cells expressing reduced levels of USP1 show a higher level of ubiquitinated PCNA and an increase in point mutations upon UV irradiation. SIMILARITY: Belongs to the peptidase C19 family. SEQUENCE CAUTION: Sequence=AAH18745.1; Type=Miscellaneous discrepancy; Note=Contaminating sequence. Potential poly-A sequence; Sequence=AAH32364.1; Type=Miscellaneous discrepancy; Note=Contaminating sequence. Potential poly-A sequence; Sequence=BAD92130.1; Type=Frameshift; Positions=230;
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on O94782
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
Biological Process: GO:0001501 skeletal system development GO:0006281 DNA repair GO:0006282 regulation of DNA repair GO:0006508 proteolysis GO:0006511 ubiquitin-dependent protein catabolic process GO:0006974 cellular response to DNA damage stimulus GO:0009411 response to UV GO:0016579 protein deubiquitination GO:0035520 monoubiquitinated protein deubiquitination GO:0042769 DNA damage response, detection of DNA damage
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Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
