Target:Ubr1
Gene Name:UBR1
Protein Name:E3 ubiquitin-protein ligase UBR1
Human Gene Id:197131
Human Swiss Prot No:Q8IWV7
Mouse Gene Id:22222
Mouse Swiss Prot No:O70481
Immunogen:The antiserum was produced against synthesized peptide derived from human UBR1. AA range:821-870
Specificity:Ubr1 Polyclonal Antibody detects endogenous levels of Ubr1 protein.
Formulation:Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Source:Polyclonal, Rabbit,IgG
Dilution:WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:20000.. IF 1:50-200
Purification:The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration:1 mg/ml
Storage Stability:-15°C to -25°C/1 year(Do not lower than -25°C)
Other Name:UBR1;E3 ubiquitin-protein ligase UBR1;N-recognin-1;Ubiquitin-protein ligase E3-alpha-1;Ubiquitin-protein ligase E3-alpha-I
Observed Band(KD):200kD
Background: The N-end rule pathway is one proteolytic pathway of the ubiquitin system. The recognition component of this pathway, encoded by this gene, binds to a destabilizing N-terminal residue of a substrate protein and participates in the formation of a substrate-linked multiubiquitin chain. This leads to the eventual degradation of the substrate protein. The protein described in this record has a RING-type zinc finger and a UBR-type zinc finger. Mutations in this gene have been associated with Johanson-Blizzard syndrome. [provided by RefSeq, Jul 2008],
Function:developmental stage:Expressed in fetal pancreas.,disease:Defects in UBR1 are a cause of Johanson-Blizzard syndrome (JBS) [MIM:243800]. This disorder includes congenital exocrine pancreatic insufficiency, multiple malformations such as nasal wing aplasia, and frequent mental retardation. Pancreas of individuals with JBS do not express UBR1 and show intrauterine-onset destructive pancreatitis.,domain:The RING-H2 zinc finger is an atypical RING finger with a His ligand in place of the fourth Cys of the classical motif.,function:E3 ubiquitin-protein ligase which is a component of the N-end rule pathway. Recognizes and binds to proteins bearing specific N-terminal residues that are destabilizing according to the N-end rule, leading to their ubiquitination and subsequent degradation. May be involved in pancreatic homeostasis.,pathway:Protein modification; protein ubiquitination.,PTM:Phosphoryl
Subcellular Location:Cytoplasm, cytosol .
Expression:Broadly expressed, with highest levels in skeletal muscle, kidney and pancreas. Present in acinar cells of the pancreas (at protein level).