Von Hippel Lindau/VHL Antibody [A6D6]

Catalog No.: F2577

Application: Reactivity: Human

Lane 1: HEK-293T (transfected with pCMV6-ENTRY control cDNA), Lane 2: HEK-293T (transfected with pCMV6-ENTRY Von Hippel Lindau cDNA)

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代表番号: 045-509-1970|電子メール：sales@selleck.co.jp

使用情報

Dilution
WB1:4000 IHC1:150

Application
WB, IHC

Source
Mouse Monoclonal Antibody

Reactivity
Human

Storage Buffer
PBS, pH 7.2+50% Glycerol+0.05% BSA+0.01% NaN3

Storage (from the date of receipt)
-20°C (avoid freeze-thaw cycles), 2 years

Predicted MW
24 kDa

Datasheet & SDS

生物学的記述

Specificity
Von Hippel Lindau/VHL Antibody [A6D6] detects endogenous levels of total Von Hippel Lindau/VHL protein.

Clone
A6D6

Synonym(s)
von Hippel-Lindau disease tumor suppressor; Protein G7; pVHL; VHL

Background
The Von Hippel Lindau/VHL gene, located on chromosome 3p25, is a classical tumor suppressor gene whose inactivation underlies the inherited VHL cancer syndrome and most sporadic clear cell renal cell carcinomas. It encodes the pVHL protein, a small protein expressed widely in fetal and adult tissues, with particularly strong functional relevance in renal proximal tubule cells—the precursors of clear cell renal cancer. Structurally, pVHL contains an α-domain that binds ElonginC/ElonginB and Cul2 to form a multiprotein E3 ubiquitin ligase complex, and a β-domain that recognizes substrates, such as the hypoxia-inducible factors (HIF-1α and HIF-2α), targeting them for ubiquitin-mediated proteasomal degradation. Beyond HIF regulation, pVHL interacts with proteins such as Jade-1, PKCλ, fibronectin, and RNA-binding factors, linking it to diverse functions in oxygen sensing, angiogenesis control, extracellular matrix assembly, transcriptional regulation, cell cycle arrest, apoptosis inhibition, and cellular differentiation. Loss of VHL function results in HIF stabilization, VEGF overproduction, and abnormal vascular growth, driving tumorigenesis. Thus, VHL acts as a molecular “gatekeeper” in renal carcinogenesis, with structural domains specialized for multiprotein complex assembly and substrate recognition, and its broad expression and versatile functions explain the phenotypic heterogeneity of VHL disease.

Background

The Von Hippel Lindau/VHL gene, located on chromosome 3p25, is a classical tumor suppressor gene whose inactivation underlies the inherited VHL cancer syndrome and most sporadic clear cell renal cell carcinomas. It encodes the pVHL protein, a small protein expressed widely in fetal and adult tissues, with particularly strong functional relevance in renal proximal tubule cells—the precursors of clear cell renal cancer. Structurally, pVHL contains an α-domain that binds ElonginC/ElonginB and Cul2 to form a multiprotein E3 ubiquitin ligase complex, and a β-domain that recognizes substrates, such as the hypoxia-inducible factors (HIF-1α and HIF-2α), targeting them for ubiquitin-mediated proteasomal degradation. Beyond HIF regulation, pVHL interacts with proteins such as Jade-1, PKCλ, fibronectin, and RNA-binding factors, linking it to diverse functions in oxygen sensing, angiogenesis control, extracellular matrix assembly, transcriptional regulation, cell cycle arrest, apoptosis inhibition, and cellular differentiation. Loss of VHL function results in HIF stabilization, VEGF overproduction, and abnormal vascular growth, driving tumorigenesis. Thus, VHL acts as a molecular “gatekeeper” in renal carcinogenesis, with structural domains specialized for multiprotein complex assembly and substrate recognition, and its broad expression and versatile functions explain the phenotypic heterogeneity of VHL disease.

References
[1] Zhou MI, et al. J Biol Chem. 2002 Oct 18;277(42):39887-98. [2] Stebbins CE, et al. Science. 1999 Apr 16;284(5413):455-61.

PVDFメンブレン孔径参考表

Protein Size (kDa)	PVDF Transfer Membrane Pore Size (μm)
< 10	0.22
10-20	0.22
20-30	0.45
30-45	0.45
45-70	0.45
80-100	0.45
100-140	0.45
> 140	0.45

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Protein Size (kDa)	Separating Gel Percentage
4-40	20%
12-45	15%
10-70	12.5%
15-100	10%
25-100	8%
60-210	5%