|Application ||WB, IHC-P, IF, FC, E|
|Other Accession||Q64259, P40338|
|Calculated MW||24153 Da|
|Antigen Region||43-71 aa|
|Other Names||Von Hippel-Lindau disease tumor suppressor, Protein G7, pVHL, VHL|
|Target/Specificity||This VHL antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 43-71 amino acids from the N-terminal region of human VHL.|
|Format||Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.|
|Storage||Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||VHL Antibody (N-term) is for research use only and not for use in diagnostic or therapeutic procedures.|
|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.|
|Cellular 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|
|Tissue Location||Expressed in the adult and fetal brain and kidney|
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Von Hippel-Lindau syndrome (VHL) is a dominantly inherited familial cancer syndrome predisposing to a variety of malignant and benign tumors. A germline mutation of VHL gene is the basis of familial inheritance of VHL syndrome. The protein is a component of the protein complex that includes elongin B, elongin C, and cullin-2, and possesses ubiquitin ligase E3 activity. This 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. RNA polymerase II subunit POLR2G/RPB7 is also reported to be a target of this protein.
Olmos,G., Cell. Mol. Life Sci. 66 (13), 2167-2180 (2009) Hatzimichael,E., Clin Lymphoma Myeloma 9 (3), 239-242 (2009) Luu,V.D., Clin. Cancer Res. 15 (10), 3297-3304 (2009)
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