MA5-13940 targets von Hippel-Lindau Protein in immunohistochemistry (paraffin) and immunofluorescence applications and shows reactivity with Human and mouse samples.
The MA5-13940 immunogen is a recombinant protein containing aa 1-213 of human VHL.
Molecular genetic studies suggest that VHL is a tumor-suppressor gene. 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. 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.
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Protein Aliases: elongin binding protein; Protein G7; pVHL; von Hippel-Lindau disease tumor suppressor; von Hippel-Lindau syndrome homolog; von Hippel-Lindau syndrome protein homolog; von Hippel-Lindau tumor suppressor, E3 ubiquitin protein ligase
Gene Aliases: HRCA1; pVHL; RCA1; VHL; VHL1; Vhlh
Molecular Function: ligase