Signal Transduction Antibodies 1
Anti-VHL Antibody (CAB11240)
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- SKU:
- CAB11240
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Signal Transduction
Description
| Antibody Name: | Anti-VHL Antibody |
| Antibody SKU: | CAB11240 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB |
| Reactivity: | Human, Mouse |
| Host Species: | Rabbit |
| Immunogen: | A synthetic peptide corresponding to a sequence within amino acids 1-100 of human VHL (NP_937799.1). |
| Application: | WB |
| Recommended Dilution: | WB 1:500 - 1:2000 |
| Reactivity: | Human, Mouse |
| Positive Samples: | Mouse brain, Mouse kidneyMCF-7, Raji, Mouse kidney |
| Immunogen: | A synthetic peptide corresponding to a sequence within amino acids 1-100 of human VHL (NP_937799.1). |
| Purification Method: | Affinity purification |
| Storage Buffer: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | MPRR AENW DEAE VGAE EAGV EEYG PEED GGEE SGAE ESGP EESG PEEL GAEE EMEA GRPR PVLR SVNS REPS QVIF CNRS PRVV LPVW LNFD GEPQ PYPT |
| Gene ID: | 7428 |
| Uniprot: | P40337 |
| Cellular Location: | Cytoplasm, Membrane, Nucleus, Nucleus, Peripheral membrane protein |
| Calculated MW: | 18kDa/19kDa/24kDa |
| Observed MW: | 18kDa, 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 |
 | Western blot analysis of extracts of various cell lines, using VHL antibody (CAB11240) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s. |
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