Search
E3 ubiquitin-protein ligase UBR1; N-recognin-1; ubiquitin-protein ligase E3-alpha-1; ubiquitin-protein ligase E3-alpha-I (UBR1)
Function:
1) ubiquitin ligase protein, ubiquitin conjugation, 3rd step
2) component of the N-end rule pathway
3) recognizes & binds to proteins bearing specific amino- terminal residues that are destabilizing according to the N-end rule, leading to ubiquitination & subsequent degradation
4) pancreatic homeostasis
5) interacts with RECQL4
Structure:
- the RING-H2 Zn+2 finger is an atypical RING finger with a His ligand in place of the fourth Cys of the classical motif
- belongs to the UBR1 family
- contains 1 RING-type Zn+2 finger
- contains 1 UBR-type Zn+2 finger
Compartment: cytoplasm; cytosol
Alternative splicing: named isoforms=2
Expression:
- broadly expressed
- highest levels in skeletal muscle, kidney, pancreas, acinar cells of pancreas, fetal pancreas
Pathology:
- defects in UBR1 are a cause of Johanson-Blizzard syndrome
General
phosphoprotein
E3 ubiquitin ligase; ubiquitin-ligating enzyme E3; N end-recognizing protein
zinc finger protein
Properties
SIZE: entity length = 1749 aa
MW = 200 kD
COMPARTMENT: cytoplasm
MOTIF: Thr phosphorylation site {T21}
Zinc finger
NAME: Zinc finger
SITE: 97-168
EFFECTOR-BOUND: Zn+2
proline-rich region
SITE: 864-869
MOTIF: proline residue (SEVERAL)
RING-finger {1098-1201}
MOTIF: Ser phosphorylation site {S1179}
EFFECTOR-BOUND: Zn+2
FOR-BINDING-OF: DNA motif
Database Correlations
OMIM correlations
MORBIDMAP 605981
UniProt Q8IWV7
PFAM correlations
Entrez Gene 197131
Kegg hsa:197131
References
UniProt :accession Q8IWV7