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. Binds leucine and is a negative regulator of the leucine-mTOR signaling pathway, thereby controlling cell growth.
Broadly expressed, with highest levels in skeletal muscle, kidney and pancreas. Present in acinar cells of the pancreas (at protein level).
Protein modification; protein ubiquitination.
Involvement in 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.
Belongs to the UBR1 family. Contains 1 RING-type zinc finger. Contains 1 UBR-type zinc finger.
Expressed in fetal pancreas.
The RING-H2 zinc finger is an atypical RING finger with a His ligand in place of the fourth Cys of the classical motif.
Phosphorylated 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
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.