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Home / Primary Antibodies / IHC Antibodies

VHL Antibody

SKU : BHA10557123

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CUSABIO TECHONOLOGY LLC

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Mfr.No.	CSB-PA025852KA01HU
Alternative Names	Von Hippel-Lindau disease tumor suppressor; pVHL; Protein G7; VHL
Categories	Primary Antibodies
Description	The Von Hippel-Lindau (VHL) protein is a substrate recognition component of an E3 ubiquitin ligase complex containing elongin BC (TCEB1 and TCEB2), cullin 1 (CUL1), and RING-box protein 1 (RBX1). VHL protein has been shown to exist as three distinct isoforms resulting from alternatively spliced transcript variants. Loss of VHL protein function results in a dominantly inherited familial cancer syndrome that manifests as angiomas of the retina, hemangioblastomas of the central nervous system, renal clear-cell carcinomas and pheochromocytomas. Under normoxic conditions, VHL directs the ubiquitylation and subsequent proteosomal degradation of the hypoxia inducible factor HIF alpha, maintaining very low levels of HIF alpha in the cell. Cellular exposure to hypoxic conditions, or loss of VHL protein function, results in increased HIF alpha protein levels and increased expression of HIF-induced gene products, many of which are angiogenesis factors such as vascular endothelial growth factor (VEGF). Thus, loss of VHL protein function is believed to contribute to the formation of highly vascular neoplasias. In addition to HIF alpha, VHL is known to regulate the ubiquitylation of several other proteins, including tat-binding protein 1 (TBP-1), the atypical protein kinase C lambda (aPKC), and two subunits of the multiprotein RNA Polymerase II complex (RPB1 and RPB7). Interactions with elongin BC, RPB1, RPB7 and the pVHL-associated KRAB-A domain containing protein (VHLaK) suggest that VHL may also play a more direct role in transcriptional repression.
Hgnc	12687
Host	Rabbit
Immunogen	Recombinant protein of human VHL
Intended Use	For research use only. Not for human, diagnostic or therapeutic use.
Involvement In Disease	Pheochromocytoma (PCC); von Hippel-Lindau disease (VHLD); Erythrocytosis, familial, 2 (ECYT2); Renal cell carcinoma (RCC)
Isotype	IgG
Mgi	103223
Raised In	Rabbit
Reactivity	Human, Mouse, Rat
Regulatory	RUO
Relevance	The Von Hippel-Lindau (VHL) protein is a substrate recognition component of an E3 ubiquitin ligase complex containing elongin BC (TCEB1 and TCEB2), cullin 1 (CUL1), and RING-box protein 1 (RBX1). VHL protein has been shown to exist as three distinct isoforms resulting from alternatively spliced transcript variants. Loss of VHL protein function results in a dominantly inherited familial cancer syndrome that manifests as angiomas of the retina, hemangioblastomas of the central nervous system, renal clear-cell carcinomas and pheochromocytomas. Under normoxic conditions, VHL directs the ubiquitylation and subsequent proteosomal degradation of the hypoxia inducible factor HIF alpha, maintaining very low levels of HIF alpha in the cell. Cellular exposure to hypoxic conditions, or loss of VHL protein function, results in increased HIF alpha protein levels and increased expression of HIF-induced gene products, many of which are angiogenesis factors such as vascular endothelial growth factor (VEGF). Thus, loss of VHL protein function is believed to contribute to the formation of highly vascular neoplasias. In addition to HIF alpha, VHL is known to regulate the ubiquitylation of several other proteins, including tat-binding protein 1 (TBP-1), the atypical protein kinase C lambda (aPKC), and two subunits of the multiprotein RNA Polymerase II complex (RPB1 and RPB7). Interactions with elongin BC, RPB1, RPB7 and the pVHL-associated KRAB-A domain containing protein (VHLaK) suggest that VHL may also play a more direct role in transcriptional repression.
Size	100μl(100μg)
Species	Homo Sapiens (Human)
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, SUBCELLULAR LOCATION: Isoform 3: Cytoplasm, Nucleus

Rgd	3960
Target Details	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 this gene is the basis of familial inheritance of VHL syndrome. This 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. Alternatively spliced transcript variants encoding distinct isoforms have been observed.
Uniprot	P40337
Database Links	HGNC:12687 OMIM:144700 KEGG:hsa:7428 STRING:9606.ENSP00000256474 UniGene:Hs.517792
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.
Pathway	HIF-1 signaling pathway Ubiquitin mediated proteolysis
Protein Families	VHL family
Tissue Specificity	Expressed in the adult and fetal brain and kidney.

Buffer	Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Format	liquid
Purification	Affinity purification
Purity	Affinity purification
Storage	Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Storage Buffer	Store at -20oC or -80oC. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Applications

ELISA, WB, IHC

Description

ELISA ; WB ; IHC, WB ; ELISA ; IHC

Dilution

WB	1:500-1:2000
IHC	1:50-1:200

Tested Application

ELISA,WB,IHC;WB:1:500-1:2000,IHC:1:50-1:200

Datasheet
ELISA Protocol
Western Blotting(WB) Protocol
Immunohistochemistry (IHC) Protocol

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SKU	Supplier No.	Size	Your price	Quantity
BHA10557123	CSB-PA025852KA01HU	100μl	$387.33		Add to Cart

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