FunctionE3 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. Binds leucine and is a negative regulator of the leucine-mTOR signaling pathway, thereby controlling cell growth.
Tissue specificityBroadly expressed, with highest levels in skeletal muscle, kidney and pancreas. Present in acinar cells of the pancreas (at protein level).
PathwayProtein modification; protein ubiquitination.
Involvement in diseaseDefects 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.
Sequence similaritiesBelongs to the UBR1 family. Contains 1 RING-type zinc finger. Contains 1 UBR-type zinc finger.
Developmental stageExpressed in fetal pancreas.
DomainThe RING-H2 zinc finger is an atypical RING finger with a His ligand in place of the fourth Cys of the classical motif.
Post-translational modificationsPhosphorylated upon DNA damage, probably by ATM or ATR.
ubiquitin protein ligase E3 component n-recognin 1 antibody
Ubiquitin-protein ligase E3-alpha-1 antibody
Ubiquitin-protein ligase E3-alpha-I antibody
Anti-UBR1 antibody images
Western blot - UBR1 antibody (ab108215)
All lanes : Anti-UBR1 antibody (ab108215) at 0.1 µg/ml
Lane 1 : Jurkat whole cell lysate at 50 µg Lane 2 : Jurkat whole cell lysate at 15 µg Lane 3 : Jurkat whole cell lysate at 5 µg
Developed using the ECL technique
Predicted band size : 200 kDa
Exposure time : 3 minutes
Immunoprecipitation - UBR1 antibody (ab108215)
Detection of UBR1 by Western Blot of Immunprecipitate.
Lane 1: ab108215 at 1µg/ml staining UBR1 in Jurkat whole cell lysate immunoprecipitated using ab108215 at 6µg/mg lysate (1 mg/IP; 20% of IP loaded/lane). Lane 2: Control IgG
Detection: Chemiluminescence with exposure time of 30 seconds.
References for Anti-UBR1 antibody (ab108215)
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