Product Overview
Description
CLPP-00151038 is recombinant human RANBP2 protein, Active
Applications
Functional Studies, SDS-PAGE
Protein Length
Protein fragment
Nature
Recombinant Protein
Sequence
LKSNNSETSSVAQSGSESKVEPKKCELSKNSDIEQSSDSKVKNLFASFPTEESSINYTFKTPEKAKEKKKPEDSPSDDDVLIVYELTPTAEQKALATKLKLPPTFFCYKNRPDYVSEEEEDDEDFETAVKKLNGKLYLDGSEKCRPLEENTADNEKECIIVWEKKPTVEEKAKADTLKLPPTFFCGVCSDTDEDNGNGEDFQSELQKVQEAQKSQTEEITSTTDSVYTGGTEVMVPSFCKSEEPDSITKSISSPSVSSETMDKPVDLSTRKEIDTDSTSQGESKIV
Sequence Similarities
Contains 1 PPIase cyclophilin-type domain. Contains 4 RanBD1 domains. Contains 8 RanBP2-type zinc fingers. Contains 1 TPR repeat.
Target Information
Alternative Names
358 kDa nucleoporin; ANE1; E3 SUMO-protein ligase RanBP2; IIAE3; Nuclear pore complex protein Nup358; Nucleoporin 358; Nucleoporin Nup358; NUP358; p270; RAN binding protein 2; Ran-binding protein 2; RANBP2; RBP2_HUMAN; Transformation related protein 2; TRP 1; TRP 2; TRP1; TRP2
Protein 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. Binds single-stranded RNA (in vitro). May bind DNA. Component of the nuclear export pathway. Specific docking site for the nuclear export factor exportin-1. Sumoylates PML at 'Lys-490' which is essential for the proper assembly of PML-NB. Recruits BICD2 to the nuclear envelope and cytoplasmic stacks of nuclear pore complex known as annulate lamellae during G2 phase of cell cycle. Probable inactive PPIase with no peptidyl-prolyl cis-trans isomerase activity.
Involvement in Disease
Defects in RANBP2 are the cause of susceptibility to encephalopathy acute necrotizing type 1 (ANE1). A rapidly progressive encephalopathy manifesting in susceptibile individuals with seizures and coma. It 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 characteristic symmetric lesions present in the thalami, pons and brainstem.
Shipping & Handling
Constituents
0.82% Sodium phosphate, 1.74% Sodium chloride, 0.002% PMSF, 0.004% DTT, 25% Glycerol (glycerin, glycerine).
Shipping
Shipped on Dry Ice.
