Synthesized peptide derived from human RBP2 AA range: 108-158

This antibody detects endogenous levels of RBP2 at Human/Mouse

RANBP2 NUP358

RBP2

RAN is a small GTP-binding protein of the RAS superfamily that is associated with the nuclear membrane and is thought to control a variety of cellular functions through its interactions with other proteins. This gene encodes a very large RAN-binding protein that immunolocalizes to the nuclear pore complex. The protein is a giant scaffold and mosaic cyclophilin-related nucleoporin implicated in the Ran-GTPase cycle. The encoded protein directly interacts with the E2 enzyme UBC9 and strongly enhances SUMO1 transfer from UBC9 to the SUMO1 target SP100. These findings place sumoylation at the cytoplasmic filaments of the nuclear pore complex and suggest that, for some substrates, modification and nuclear import are linked events. This gene is partially duplicated in a gene cluster that lies in a hot spot for recombination on chromosome 2q. [provided by RefSeq, Jul 2008],

Disease:Defects in RANBP2 are the cause of susceptibility to acute necrotizing encephalopathy type 1 (ANE1) [MIM:608033]. Acute necrotizing encephalopathy (ANE) is a rapidly progressive encephalopathy, seizures, and coma that can occur within days in otherwise healthy children after common viral infections such as influenza and parainfluenza, without evidence of viral infection of the brain or inflammatory cell infiltration. Brain T2-weighted magnetic resonance imaging reveals characteritic symmetric lesions present in the thalami, pons, and brainstem.,Domain:Contains F-X-F-G repeats.,Function:E3 SUMO-protein ligase which facilitates SUMO1 and SUMO2 conjugation by UBE2I. Involved in transport factor (Ran-GTP, karyopherin)-mediated protein import via the F-G repeat-containing domain which acts as a docking site for substrates. Could also have isomerase or chaperone activity and may bind RNA or DNA. Component of the nuclear export pathway. Specific docking site for the nuclear export factor exportin-1.,pathway:Protein modification; protein sumoylation.,PTM:Polyubiquitinated by PARK2, which leads to proteasomal degradation.,similarity:Contains 1 PPIase cyclophilin-type domain.,similarity:Contains 1 TPR repeat.,similarity:Contains 4 RanBD1 domains.,similarity:Contains 8 RanBP2-type zinc fingers.,subcellular location:Cytoplasmic filaments.,subunit:Forms a tight complex with RANBP1 and UBE2I. Interacts with SUMO1 but not SUMO2. Interacts with PARK2. Interacts with sumoylated RANGAP1.,

Nucleus . Nucleus membrane . Nucleus, nuclear pore complex . Nucleus envelope . Detected in diffuse and discrete intranuclear foci (PubMed:11839768). Cytoplasmic filaments (PubMed:7775481). .

>>Nucleocytoplasmic transport ;
>>Viral life cycle - HIV-1 ;
>>Amyotrophic lateral sclerosis