Rabbit anti-Human VHL Polyclonal Antibody

Uniprot Id

P40337

Target Species

Human

Target Name

VHL

Target Full Name

von Hippel-Lindau disease tumor suppressor

Target Function

Involved in the ubiquitination and subsequent proteasomal degradation via the von Hippel-Lindau ubiquitination complex. Seems to act as a target recruitment subunit in the E3 ubiquitin ligase complex and recruits hydroxylated hypoxia-inducible factor (HIF) under normoxic conditions. Involved in transcriptional repression through interaction with HIF1A, HIF1AN and histone deacetylases. Ubiquitinates, in an oxygen-responsive manner, ADRB2.

Target Involvement

Pheochromocytoma (PCC); von Hippel-Lindau disease (VHLD); Erythrocytosis, familial, 2 (ECYT2); Renal cell carcinoma (RCC)

Target Subcellular Location

[Isoform 1]: Cytoplasm. Membrane; Peripheral membrane protein. Nucleus. Note=Found predominantly in the cytoplasm and with less amounts nuclear or membrane-associated. Colocalizes with ADRB2 at the cell membrane.; [Isoform 3]: Cytoplasm. Nucleus. Note=Equally distributed between the nucleus and the cytoplasm but not membrane-associated.

Target Protein Families

VHL family

Target Tissue Specificity

Expressed in the adult and fetal brain and kidney.

Target Research Area

Cancer

Target Synonyms

Elongin binding protein; G7 protein; HRCA 1; HRCA1; Protein G7; pVHL; RCA 1; RCA1; VHL 1; VHL; VHL_HUMAN; VHL1; VHLH; Von Hippel Lindau; Von Hippel Lindau disease tumor suppressor; von Hippel Lindau syndrome; von Hippel Lindau tumor suppressor; Von Hippel Lindau tumor suppressor, E3 ubiquitin protein ligase; Von Hippel-Lindau disease tumor suppressor

Target Background

This gene encodes a component of a ubiquitination complex. The encoded protein is involved in the ubiquitination and degradation of hypoxia-inducible-factor (HIF), which is a transcription factor that plays a central role in the regulation of gene expression by oxygen. In addition to oxygen-related gene expression, this protein plays a role in many other cellular processes including cilia formation, cytokine signaling, regulation of senescence, and formation of the extracellular matrix. Variants of this gene are associated with von Hippel-Lindau syndrome, pheochromocytoma, erythrocytosis, renal cell carcinoma, and cerebellar hemangioblastoma.