VHL antibody has been tested by ELISA and Western blot analysis to assure specificity and reactivity. Recommended dilution range for Western blot analysis is 1:500 ~ 1,000. Recommended starting dilution is 1:500.
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).
[1] Kibel, A. et al. (1995) Science 269, 1444-6.
[2] Pause, A. et al. (1997) Proc Natl Acad Sci U S A 94, 2156-61.
[3] Kamura, T. et al. (2000) Proc Natl Acad Sci U S A 97, 10430-5.
[4] Czyzyk-Krzeska, M.F. and Meller, J. (2004) Trends Mol Med 10, 146-9.
[5] Corn, P.G. et al. (2003) Nat Genet 35, 229-37.
[6] Na, X. et al. (2003) EMBO J 22, 4249-59.
[7] Kuznetsova, A.V. et al. (2003) Proc Natl Acad Sci U S A 100, 2706-11.
[8] Li, Z. et al. (2003) EMBO J 22, 1857-1867.
