VHL Antibody

Von Hippel-Lindau Protein, Mouse Anti Human
Cat. No.
BT2839
Source
Synonyms
Von Hippel-Lindau disease tumor suppressor, pVHL, Protein G7, VHL, RCA1, VHL1, HRCA1.
Appearance
Sterile filtered colorless solution.
Purity
Usage
THE BioTek's products are furnished for LABORATORY RESEARCH USE ONLY. The product may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.
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Description

Product Specs

Introduction
Von Hippel-Lindau disease is a dominant inherited syndrome characterized by the predisposition to develop various kinds of benign and malignant tumors, including clear cell renal carcinomas, pheochromocytomas and hemangioblastomas of the central nervous system and retina. VHL syndrome is caused by germline mutation in the VHL tumor suppressor, and VHL tumors are associated with loss or mutation of the remaining wild-type allele. VHL has two domains: a roughly 100-residue NH2-terminal domain rich in β sheet (β-domain) and a smaller α-helical domain (α-domain), held together by two linkers and a polar interface. VHL protein is also involved in the degradation of hypoxia-inducible factor (HIF).
Physical Appearance
Sterile filtered colorless solution.
Formulation
1mg/ml containing PBS, pH-7.4, & 0.1% Sodium Azide.
Storage Procedures
For periods up to 1 month store at 4°C, for longer periods of time, store at -20°C. Prevent freeze thaw cycles.
Stability / Shelf Life
12 months at -20°C. 1 month at 4°C.
Applications
VHL antibody has been tested by ELISA and Western blot analysis to assure specificity and reactivity. Since application varies, however, each investigation should be titrated by the reagent to obtain optimal results. Recommended dilution range for Western blot analysis is 1:500 ~ 1,000. Recommended starting dilution is 1:500.
Synonyms
Von Hippel-Lindau disease tumor suppressor, pVHL, Protein G7, VHL, RCA1, VHL1, HRCA1.
Purification Method
VHL antibody was purified from mouse ascitic fluids by protein-G affinity chromatography.
Type
Mouse Anti Human Monoclonal.
Clone
P52A11AT.
Immunogen
Anti-human VHL mAb is derived from hybridization of mouse SP2/O myeloma cells with spleen cells from BALB/c mice immunized with recombinant human VHL amino acids 1-154 purified from E. coli.
Ig Subclass
Mouse IgG2b heavy chain and κ light chain.

Product Science Overview

Introduction

The Von Hippel-Lindau (VHL) protein is a crucial tumor suppressor protein encoded by the VHL gene. This protein plays a significant role in the regulation of cellular responses to oxygen levels. Mutations in the VHL gene are associated with Von Hippel-Lindau disease, a rare genetic disorder characterized by the development of tumors and cysts in various parts of the body .

Function of VHL Protein

The primary function of the VHL protein is to act as the substrate recognition component of an E3 ubiquitin ligase complex. This complex is responsible for the ubiquitination and subsequent degradation of hypoxia-inducible factors (HIFs), which are transcription factors that regulate gene expression in response to oxygen levels . Under normal oxygen conditions, the VHL protein binds to HIF1α, leading to its degradation. However, under hypoxic conditions, this binding does not occur, allowing HIF1α to activate the transcription of genes involved in angiogenesis and other processes .

Von Hippel-Lindau Disease

Von Hippel-Lindau disease is caused by mutations in the VHL gene, leading to the production of dysfunctional VHL protein. This results in the accumulation of HIFs and the uncontrolled growth of cells, forming tumors and cysts in various organs, including the brain, spinal cord, retina, kidneys, pancreas, and adrenal glands . The disease can be inherited or occur spontaneously, and its clinical manifestations depend on the location and size of the tumors .

Mouse Anti Human VHL Protein Antibody

The Mouse Anti Human VHL Protein antibody is a monoclonal antibody used in research to study the VHL protein. This antibody is specifically designed to bind to the human VHL protein, allowing researchers to detect and quantify its presence in various biological samples. The use of this antibody is crucial in understanding the role of VHL protein in cellular processes and its implications in diseases like Von Hippel-Lindau disease .

Applications in Research

The Mouse Anti Human VHL Protein antibody is widely used in various research applications, including:

  • Western Blotting: To detect the presence and quantify the levels of VHL protein in cell and tissue samples.
  • Immunohistochemistry: To visualize the localization of VHL protein within tissues.
  • Immunoprecipitation: To isolate and study the VHL protein and its interacting partners.
  • Flow Cytometry: To analyze the expression of VHL protein in different cell populations .

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