Anti-VHL Antibody (CAB0377)
- SKU:
- CAB0377
- Product type:
- Antibody
- Reactivity:
- Human
- Mouse
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Signal Transduction
Frequently bought together:
Description
| Antibody Name: | Anti-VHL Antibody |
| Antibody SKU: | CAB0377 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB IHC IF |
| Reactivity: | Human, Mouse, Rat |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-172 of human VHL (NP_937799.1). |
| Application: | WB IHC IF |
| Recommended Dilution: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200 |
| Reactivity: | Human, Mouse, Rat |
| Positive Samples: | HeLa, Mouse brain, Rat brain |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-172 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 LPPG TGRR IHSY RVYT LKER CLQV VRSL VKPE NYRR LDIV RSLY EDLE DHPN VQKD LERL TQER IAHQ RMGD |
| 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 |
 | Western blot analysis of extracts of various cell lines, using VHL antibody (CAB0377) 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: 30s. |
 | Immunohistochemistry of paraffin-embedded rat ovary using VHL antibody (CAB0377) at dilution of 1:200 (40x lens). |
 | Immunohistochemistry of paraffin-embedded human tonsil using VHL antibody (CAB0377) at dilution of 1:200 (40x lens). |
 | Immunohistochemistry of paraffin-embedded mouse spleen using VHL antibody (CAB0377) at dilution of 1:200 (40x lens). |
 | Immunofluorescence analysis of U-251 MG cells using VHL Polyclonal Antibody (CAB0377) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining. |
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