• 靶点：
• Ubr1

• 基因名称：
• UBR1

• 蛋白名称：
• E3 ubiquitin-protein ligase UBR1

• Human Gene Id：
• 197131

• Human Swiss Prot No：
• Q8IWV7

• Mouse Gene Id：
• 22222

• Mouse Swiss Prot No：
• O70481

• 免疫原：
• The antiserum was produced against synthesized peptide derived from human UBR1. AA range:821-870

• 特异性：
• Ubr1 Polyclonal Antibody detects endogenous levels of Ubr1 protein.

• 组成：
• Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

• 来源：
• Polyclonal, Rabbit,IgG

• 稀释：
• WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:20000.. IF 1:50-200

• 纯化工艺：
• The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

• 浓度：
• 1 mg/ml

• 储存：
• -15°C to -25°C/1 year(Do not lower than -25°C)

• 其他名称：
• UBR1;E3 ubiquitin-protein ligase UBR1;N-recognin-1;Ubiquitin-protein ligase E3-alpha-1;Ubiquitin-protein ligase E3-alpha-I

• 实测条带：
• 200kD

• 背景：
• 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],

• 功能：
• 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

• 细胞定位：
• Cytoplasm, cytosol .

• 组织表达：
• 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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