VBP1
Description
The VBP1 (VHL binding protein 1) is a protein-coding gene located on chromosome X.
Prefoldin subunit 3 (VBP-1), also known as Von Hippel–Lindau binding protein 1, is a prefoldin chaperone protein that binds to von Hippel–Lindau protein and transports it from perinuclear granules to the nucleus or cytoplasm inside the cell. It is also involved in transporting nascent polypeptides to cytosolic chaperonins for post-translational folding. VBP-1 is a 197–amino acid heterohexamer comprising two prefoldin-α and four prefoldin-β subunits, and is a member of the prefoldin-α subunit family. It is ubiquitously expressed in tissues, and is located in the cell nucleus and cytoplasm. The VBP1 gene is located at Xq28. Homologues are known to exist between human VBP-1 and proteins in mice, Drosophila and C. elegans.
VBP1 binds specifically to cytosolic chaperonin (c-CPN) and transfers target proteins to it. It also binds to nascent polypeptide chains, promoting their folding in environments with competing pathways for nonnative proteins.
VBP1 is also known as HIBBJ46, PFD3, PFDN3, VBP-1.
Associated Diseases
- endometrial cancer
- nonpapillary renal cell carcinoma
- cancer
- primary pulmonary hypertension
- von Hippel-Lindau disease
