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von Hippel-Lindau Protein

Catalog Number: EXA-X1479M

 

Details

It recognizes a protein of 30kDa which is specifically identified as von Hippel-Lindau protein (VHL). Epitope of this antibody localizes between amino acids 1-54 as it does not recognize the truncated form of VHL protein. Alterations in the von Hippel-Lindau (VHL) gene are correlated with a diverse group of neoplasms including hemangioblastoma, clear cell renal carcinoma (RCC), and pheochromocytoma. Molecular genetic studies suggest that VHL is a tumor-suppressor gene; correspondingly, reintroduction of a VHL complementary DNA (cDNA) into RCC cells inhibits their ability to form tumors in nude mice. Two transcriptional elongation factors, Elongin B and C, are shown to bind in vitro and in vivo to a short, colinear region of the VHL protein (pVHL) that is frequently mutated in human tumors. It is proposed that pVHL functions in vivo as a negative regulator of transcription elongation.

Additional Information

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