Molecular weight (DA)
200kD
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.
Dilution rate
WB: 1/500 - 1/2000. IHC: 1/100 - 1/300. ELISA: 1/20000.. IF 1:50-200
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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
Protein name
E3 ubiquitin-protein ligase UBR1
Other name
UBR1; E3 ubiquitin-protein ligase UBR1; N-recognin-1; Ubiquitin-protein ligase E3-alpha-1; Ubiquitin-protein ligase E3-alpha-I
Human protein sequence Database
Q8IWV7
Mouse protein sequence database
O70481
Rat protein sequence database
Cellular localization
Cytoplasm, cytosol .
Tissue expression
Broadly expressed, with highest levels in skeletal muscle, kidney and pancreas. Present in acinar cells of the pancreas (at protein level).
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