Von Hippel-Lindau disease is a dominant inherited syndrome characterized by the predisposition to develop various kinds of benign and malignant tumors， including clear cell renal carcinomas， pheochromocytomas and hemangioblastomas of the central nervous system and retina. VHL syndrome is caused by germline mutation in the VHL tumor suppressor， and VHL tumors are associated with loss or mutation of the remaining wild-type allele. VHL has two domains: a roughly 100-residue NH2-terminal domain rich in sheet （-domain） and a smaller -helical domain （-domain）， held together by two linkers and a polar interface. VHL protein is also involved in the degradation of hypoxia-inducible factor （HIF）.
应用类型
ELISA， Western blot
免疫原
Anti-human VHL mAb is derived from hybridization of mouse SP2/O myeloma cells with spleen cells from BALB/c mice immunized with recombinant human VHL amino acids 1-154 purified from E. coli.
