2024年12月25日 · RANBP2 (RAN Binding Protein 2) is a Protein Coding gene. Diseases associated with RANBP2 include Familial Acute Necrotizing Encephalopathy and Autosomal Recessive Hypohidrotic Ectodermal Dysplasia. Among its related pathways are Transport of the SLBP independent Mature mRNA and EML4 and NUDC in mitotic spindle formation.

The RanBP2 gene encodes a very large RAN-binding protein that localizes to cytoplasmic filaments emanating from the nuclear pore complex. RanBP2/Nup358 is a giant scaffold and mosaic cyclophilin -related nucleoporin implicated in …

The RANBP2 gene provides instructions for making a protein that typically associates with a protein complex known as the nuclear pore. Learn about this gene and related health conditions.

2016年5月10日 · Here, we show that the RanBP2/RanGAP1*SUMO1/Ubc9 complex functions as an autonomous disassembly machine with a preference for the export receptor Crm1.

Missense mutations in RAN Binding Protein 2 (RANBP2) can cause genetic (also known as familial) acute necrotizing encephalopathy (ANE1). Persons with RANBP2 mutations are susceptible to infection triggered attacks which can occur in a multiphasic manner, causing severe neurologic morbidity and mortality.

2024年12月23日 · This study investigated RANBP2 mutations in children with acute necrotizing encephalopathy (ANE) and conducted a systematic review of the differences in clinical characteristics between with or without RANBP2 mutations.

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.

2021年2月28日 · 该研究首次阐明了RanBP2可利用其SUMO E3连接酶活性调控miRNA沉默途径，最终实现RanBP2对IL-6和TNF-α等促炎性细胞因子的mRNA的翻译抑制，为揭示ANE1的发病机理奠定基础。

RANBP2 is the first native and positive allosteric activator known to jump-start and boost directly the activity of KIF5B. The nuclear pore component Nup358 promotes transportin-dependent nuclear import.

2024年11月26日 · Depletion of the nuclear transport factor RANBP2 by RNAi blocks HIV-1 nuclear import and integration efficiency as assayed by the appearance of circular viral DNAs, suggesting HIV-1 IN interacts with RANBP2 in cells