immunohistochemistry: 10-20 μg/mL using formalin-fixed paraffin-embedded human kidney. indirect immunofluorescence: 5-10 μg/mL using HeLa cells. western blot: 1-2 μg/mL using HEK-293T cells overexpressing human VHL.
Von Hippel-Lindau (VHL) gene encodes a 213 amino acid polypeptide. VHL interacts stably with proteins like Fibronectin, Elongin B and Elongin C, Cullin-2 and Rbx1. pVHL is shuttled from nucleus to cytoplasm depending on the transcription manner. VHL tumor suppressor gene is located on human chromosome 3p25.3. VHL disease is an autosomal dominant disorder.
Immunogen
synthetic peptide corresponding to a sequence located at the C-terminus of human VHL, conjugated to KLH. The corresponding sequence is identical in human VHL isoform 2.
Biochem/physiol Actions
Von Hippel-Lindau (VHL) is known to regulate cellular metabolism by modulating hypoxia-inducible factor (HIF-1) proteins. VHL protein (pVHL) is used as a marker for proliferative lesions. VHL disease is a hereditary cancer syndrome, which is characterized by vascular tumors. It is caused by the inactivation of VHL protein. Expression of VHL gene is deregulated in papillary thyroid cancers (PTC). Mutations in this gene causes sporadic clear cell renal carcinomas as well as von Hippel-Lindau disease, which is characterised by hemangioblastoma.
Physical form
Solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide.
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