RANBP2

RANBP2

A gene on chromosome 2q12.3 that encodes a very large RAN-binding protein that immunolocalises to the nuclear pore complex. It is a scaffold and mosaic cyclophilin-related nucleoporin involved in the Ran-GTPase cycle; it interacts directly with the E2 enzyme UBC9 and enhances the transfer of SUMO1 (sumoylation) to its target SP100. RANBP2 is partially duplicated in a gene cluster that lies in the chromosome 2 recombination hot spot.
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Localization of ALK-1 within the cell seems to be determined by its fusion partner, leading to diffuse cytoplasmic staining for ALK-1 with TPM3, TPM4, CARS, and ATIC and SEC31L1 fusion partners (all of which are cytoplasmic proteins), nuclear staining with the RANBP2 fusion partner (encoding a nuclear pore protein), and granular cytoplasmic staining with the CLTC fusion partner (encoding a structural protein of coated vesicles).
Inflammatory Myofibroblastic Tumor of the Bladder
Infection-triggered familial or recurrent cases of acute necrotizing encephalopathy caused by mutations in a component of the nuclear pore, RANBP2.
Novel human reovirus isolated from children with acute necrotizing encephalopathy
The ALK gene rearrangements identified that have led to fusion with other genes include ATIC, (41) CARS, (61,62) TPM3, (63-65) TPM4, (63,65,66) TPM3 and TPM4, (67) CLTC, (60,65,68) RANBP2, (60,69) and SEC31L1 (SEC31A).
Low-grade myofibroblastic proliferations of the urinary bladder

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