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Protein Page:
UBR1 (human)

UBR1 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. Defects in UBR1 are a cause of Johanson-Blizzard syndrome (JBS). 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. 2 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: EC 6.3.2.-; EC; Ligase; Ubiquitin conjugating system; Ubiquitin ligase
Chromosomal Location of Human Ortholog: 15q13
Cellular Component: cytoplasm; ubiquitin ligase complex
Biological Process: negative regulation of TOR signaling pathway
Disease: Johanson-blizzard Syndrome
Reference #:  Q8IWV7 (UniProtKB)
Alt. Names/Synonyms: E3 ubiquitin-protein ligase UBR1; E3a ligase; JBS; MGC142065; MGC142067; N-recognin-1; ubiquitin ligase E3 alpha-I; ubiquitin protein ligase E3 component n-recognin 1; ubiquitin-protein ligase E3-alpha; Ubiquitin-protein ligase E3-alpha-1; Ubiquitin-protein ligase E3-alpha-I; UBR1
Gene Symbols: UBR1
Molecular weight: 200,211 Da
Basal Isoelectric point: 5.67  Predict pI for various phosphorylation states
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Protein Structure Not Found.
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