null

Anti-VHL Mouse Monoclonal Antibody (CAB11872)

SKU:
CAB11872
Product Type:
Antibody
Reactivity:
Human
Mouse
Rat
Host Species:
Mouse
Isotype:
IgG
Antibody Type:
Monoclonal Antibody
Research Area:
Signal Transduction
€238
Frequently bought together:

Description

system_update_altDatasheetsystem_update_altMSDS
Antibody Name:Anti-VHL Mouse Monoclonal Antibody
Antibody SKU:CAB11872
Antibody Size:50uL
Application:WB IHC IF IP
Reactivity:Human, Mouse, Rat
Host Species:Mouse
Immunogen:Recombinant protein of human VHL
Application:WB IHC IF IP
Recommended Dilution:WB 1:500 - 1:2000 IHC 1:50 - 1:100 IF 1:50 - 1:100 IP 1:20 - 1:50
Reactivity:Human, Mouse, Rat
Positive Samples:Mouse brain, Mouse lung, Tissue extracts, RAW264.7
Immunogen:Recombinant protein of human VHL
Purification Method:Affinity purification
Storage Buffer:Store at 4?. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, pH7.3.
Isotype:IgG
Sequence:Email for sequence
Gene ID:7428
Uniprot:P40337
Cellular Location:Cytoplasm, Membrane, Nucleus, Nucleus, Peripheral membrane protein
Calculated MW:18kDa/19kDa/24kDa
Observed MW:24kDa
Synonyms:VHL, HRCA1, RCA1, VHL1, pVHL, PVHL
Background: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. The protein encoded by this gene 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 Protein Function:VHL: Involved in the ubiquitination and subsequent proteasomal degradation via the von Hippel-Lindau ubiquitination complex. Seems to act as 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. Component of the VCB (VHL-Elongin BC-CUL2) complex; this complex acts as a ubiquitin-ligase E3 and directs proteasome- dependent degradation of targeted proteins. Interacts with CUL2; this interaction is dependent on the integrity of the trimeric VBC complex. Interacts (via the beta domain) with HIF1A (via the NTAD domain); this interaction mediates degradation of HIF1A in normoxia and, in hypoxia, prevents ubiqitination and degradation of HIF1A by mediating hypoxia-induced translocation to the nucleus, a process which requires a hypoxia-dependent regulatory signal. Interacts with ADRB2; the interaction, in normoxia, is dependent on hydroxylation of ADRB2 and the subsequent VCB- mediated ubiquitination and degradation of ADRB2. Under hypoxia, hydroxylation, interaction with VHL, ubiquitination and subsequent degradation of ADRB2 are dramatically decreased. Interacts with RNF139, USP33 and PHF17. Found in a complex composed of LIMD1, VHL, EGLN1/PHD2, TCEB2 AND CUL2. Isoform 1 and isoform 3 interact with LIMD1 (via LIM zinc-binding 2), AJUBA (via LIM domains) and WTIP (via LIM domains). Interacts with EPAS1. Expressed in the adult and fetal brain and kidney. 3 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:

Protein type:Ubiquitin ligase; Tumor suppressor

Chromosomal Location of Human Ortholog: 3p25.3

Cellular Component: nucleoplasm; intermediate filament cytoskeleton; mitochondrion; membrane; endoplasmic reticulum; cytosol; nucleus

Molecular Function:protein binding; enzyme binding; ubiquitin-protein ligase activity; transcription factor binding

Biological Process: negative regulation of cell proliferation; regulation of transcription, DNA-dependent; protein stabilization; positive regulation of transcription, DNA-dependent; cell morphogenesis; protein ubiquitination; negative regulation of transcription from RNA polymerase II promoter; proteolysis; positive regulation of cell differentiation; negative regulation of apoptosis

Disease: Erythrocytosis, Familial, 2; Von Hippel-lindau Syndrome; Renal Cell Carcinoma, Nonpapillary; Pheochromocytoma

NCBI Summary: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. The protein encoded by this gene 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. [provided by RefSeq, Jul 2008]
UniProt Code:P40337
NCBI GenInfo Identifier:4033778
NCBI Gene ID:7428
NCBI Accession:P40337.2
UniProt Secondary Accession:P40337,Q13599, Q6PDA9, B2RE45,
UniProt Related Accession:P40337
Molecular Weight:Calculated: 18kDa/19kDa/24kDaObserved: 24kDa
NCBI Full Name:Von Hippel-Lindau disease tumor suppressor
NCBI Synonym Full Names:von Hippel-Lindau tumor suppressor, E3 ubiquitin protein ligase
NCBI Official Symbol:VHL  
NCBI Official Synonym Symbols:RCA1; VHL1; pVHL; HRCA1  
NCBI Protein Information:von Hippel-Lindau disease tumor suppressor; protein G7; elongin binding protein
UniProt Protein Name:Von Hippel-Lindau disease tumor suppressor
UniProt Synonym Protein Names:Protein G7; pVHL
Protein Family:Von Hippel-Lindau disease tumor suppressor
UniProt Gene Name:VHL  
UniProt Entry Name:VHL_HUMAN
Anti-VHL Mouse Monoclonal Antibody (CAB11872)Western blot analysis of extracts of various cell lines, using VHL antibody (CAB11872). Secondary antibody: HRP Goat Anti-Mouse IgG (H+L) (CABS003) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST.

Related Products