2. Gene
  3. USP1 - ubiquitin specific peptidase 1 Gene

USP1 - ubiquitin specific peptidase 1 Gene

  • Gene
  • Protein
  • Disease
  • Agent
  • Ortholog
Homo sapiens

Also known as UBP

Gene ID: 7398 | Gene type: protein coding

About USP1

Cytogenetic location: 1p31.3 Genomic coordinates (GRCh38): 1:62,436,395-62,451,804 (from NCBI)

This gene has 4 transcripts (splice variants), 211 orthologues and 71 paralogues. Broad expression in testis (RPKM 29.9), bone marrow (RPKM 17.7) and 25 other tissues.

Summary

This gene encodes a member of the ubiquitin-specific processing (UBP) family of proteases that is a deubiquitinating Enzyme (DUB) with His and Cys domains. This protein is located in the cytoplasm and cleaves the ubiquitin moiety from ubiquitin-fused precursors and ubiquitinylated proteins. The protein specifically deubiquitinates a protein in the Fanconi anemia (FA) DNA repair pathway. Alternate transcriptional splice variants have been characterized. [provided by RefSeq, Jul 2008]

USP1 Products(3)

mRNA Protein Name
NM_001017415.2 NP_001017415.1 ubiquitin carboxyl-terminal hydrolase 1
NM_001017416.2 NP_001017416.1 ubiquitin carboxyl-terminal hydrolase 1
NM_003368.5 NP_003359.3 ubiquitin carboxyl-terminal hydrolase 1
Gene Ontology
  • Molecular Function
  • Biological Process
  • Cellular Component
Molecular Function GO Annotation Evidence Reference Source
enables cysteine-type deubiquitinase activity IDA
IDA: Inferred from direct assay
18082604 GOA
enables cysteine-type deubiquitinase activity IMP
IMP: Inferred from mutant phenotype
20129063 GOA
enables peptidase activity EXP
EXP: Inferred from Experiment
16531995 GOA
enables protein binding IPI
IPI: Inferred from physical interaction
18082604 GOA
Biological Process GO Annotation Evidence Reference Source
involved in monoubiquitinated protein deubiquitination IMP
IMP: Inferred from mutant phenotype
20129063 GOA
involved in positive regulation of receptor signaling pathway via JAK-STAT IDA
IDA: Inferred from direct assay
37289831 GOA
involved in protein deubiquitination IDA
IDA: Inferred from direct assay
18082604 GOA
involved in regulation of DNA repair IDA
IDA: Inferred from direct assay
18082604 GOA
involved in response to UV IDA
IDA: Inferred from direct assay
18082604 GOA
Cellular Component GO Annotation Evidence Reference Source
located in nucleus IDA
IDA: Inferred from direct assay
18082604 GOA
EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

USP1 Protein Structure

UCH

UCH: Ubiquitin carboxyl-terminal hydrolase (81 - 600)

UCH

UCH: Ubiquitin carboxyl-terminal hydrolase (735 - 782)

  • 0
  • 200
  • 400
  • 600
  • 785 a.a.
Protein Preferred Names Protein Names

ubiquitin carboxyl-terminal hydrolase 1

Ubiquitinyl hydrolase 1, Ubiquitin carboxyl-terminal hydrolase 1, ubiquitin thiolesterase 1

USP1 Protein-protein interaction Information

Type
Protein Name Protein ID Interactor Interactor Species Interactor ID Detection Method Reference
Intra
USP1 O94782 WDR48 Homo sapiens Q8TAF3
Anti Tag CoIP
33961781
Intra
USP1 O94782 WDR48 Homo sapiens Q8TAF3
Anti Tag CoIP
28514442
Intra
USP1 O94782 WDR48 Homo sapiens Q8TAF3
Anti Tag CoIP
35271311
Intra
USP1 O94782 WDR48 Homo sapiens Q8TAF3
Anti Tag CoIP
19615732
Intra
USP1 O94782 WDR48 Homo sapiens Q8TAF3-1
Anti Tag CoIP
26388029
Cross: Cross-species interaction Intra: Intraspecies interaction

Related Diseases

Diseases Alias
Fanconi Anemia, Complementation Group I

Fanconi Anemia Complementation Group I

FANCI
Mast Syndrome

SPG21

Spastic Paraplegia 21, Autosomal Recessive

Autosomal Recessive Spastic Paraplegia Type 21

Autosomal Recessive Spastic Paraplegia 21

Hereditary Spastic Paraplegia 21
Fanconi Anemia, Complementation Group D2

Fanconi Anemia Complementation Group D2

FANCD2

Fad2

Fa4

Fancd

Fanconi Pancytopenia Type 4

Fanconi Anemia, Complementation Group D

Fanconi Pancytopenia, Type 4

Facd

Fanconi Anemia Complementation Group D
Xeroderma Pigmentosum, Variant Type

Xeroderma Pigmentosum

XPV

Xeroderma Pigmentosum Variant Type

Xeroderma Pigmentosum With Normal Dna Repair Rates

Photosensitivity With Defective Dna Synthesis

Xp

De Sanctis-Cacchione Syndrome

Desanctis-Cacchione Syndrome

Xeroderma Pigmentosa

Xerodermic Idiocy

Xeroderma Pigmentosum Variant

Xp - [Xeroderma Pigmentosum]

Atrophoderma Pigmentosum
Interstitial Nephritis, Karyomegalic

Karyomegalic Interstitial Nephritis

KMIN

Kin

Systemic Karyomegaly

Karyomegalic Tubulointerstitial Nephritis

Ktn
Aplastic Anemia

Aplastic Anemia, Susceptibility To

Anemia Aplastic

Idiopathic Aplastic Anemia

Secondary Aplastic Anemia

Idiopathic Bone Marrow Failure

Aplastic Anemia Idiopathic

AA

Anemia, Aplastic

Aplastic Anemia, Idiopathic

Erythroid Aplasia

Aa - [Aplastic Anaemia]

Haematopoietic Aplasia

Aleukia Haemorrhagica

Anaemia Due To Decreased Red Cell Production

Aplasia Bone Marrow

Aplastic Bone Marrow

Hypoplastic Anaemia Nos

Myeloid Bone Marrow Aplasia

Pancytopenia

Panhaematopenia

Hypoproliferative Anaemia

Medullary Hypoplasia

Red Blood Cells Hypoplastic Anaemia

Panmyelophthisis

Panhemocytopenia

Refractive Hypoproliferative Anaemia

Toxic Anaemia

Toxic Aplastic Anaemia

Aplastic Anaemia Due To Toxic Cause

Idiopathic Aplastic Anaemia Nos
Fanconi Anemia, Complementation Group A

Fanconi Anemia

Fanconi Pancytopenia

Fanconi Anemia Complementation Group A

FANCA

Fa

Fanconi Panmyelopathy

Fanconi'S Anemia

Fanconi Anaemia

Fanconi'S Anaemia

Fanconi Hypoplastic Anemia

Estren-Dameshek Variant Of Fanconi Anemia

Estren-Dameshek Variant Of Fanconi Pancytopenia

Fanconi Anemia Estren-Dameshek Variant

Fanconis Anemia
Diseases Alias
Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Related Agents

Pre-clinical Phase
Bioactive Molecules (3)
Cat. No. Product Name Effect
Purity Rare Diseases
HY-153731 USP1-IN-4 Inhibitor / Unkown
HY-161878 USP1-IN-10 Inhibitor / Unkown
HY-145471A KSQ-4279 (gentisate) Inhibitor / Unkown

Orthologs Information

Species Symbol Source ID
Rattus norvegicus USP1 RGD RGD:1306461
Canis familiaris USP1 VGNC VGNC:48177
Felis catus USP1 VGNC VGNC:66864
Macaca mulatta USP1 VGNC VGNC:78733
Mus musculus USP1 MGD MGI:2385198
Bos taurus USP1 VGNC VGNC:36709
Others USP1 NCBI