PVHL Antibody (OASL01235)

Catalog No: OASL01235

Size:50ul

Immunohistochemical analysis of paraffin-embedded Kindey. 1, Antibody was diluted at 1:200 (4C, overnight) . 2, Tris-EDTA, pH8.0 was used for antigen retrieval.

Immunohistochemical analysis of paraffin-embedded Kindey. 1, Antibody was diluted at 1:200 (4C, overnight) . 2, Tris-EDTA, pH8.0 was used for antigen retrieval.

Product Info

• Product Format: Liquid. PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
• Clonality: Monoclonal
• Isotype: IgG2b, Kappa
• Host: Mouse
• Application: IHC-P
• Additional Information: Background: von Hippel-Lindau tumor suppressor(VHL) Homo sapiens 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.
  Function: disease: defects in VHL are a cause of pheochromocytoma [MIM: 171300]. The pheochromocytomas are catecholamine-producing, chromaffin tumors that arise in the adrenal medulla in 90% of cases. In the remaining 10% of cases, they develop in extra-adrenal sympathetic ganglia and may be referred to as "paraganglioma." Pheochromocytoma usually presents with hypertension. Approximately 10% of pheochromocytoma is hereditary. The genetic basis for most cases of non-syndromic familial pheochromocytoma is unknown, disease: defects in VHL are a cause of renal cell carcinoma type 1 (RCC1) [MIM: 144700]; also called hypernephroma or adenocarcinoma of kidney. Familial renal cell carcinoma syndromes form a group of diseases characterized by a predisposition to development of renal cell carcinomas (RCCs) with various histological subtypes, disease: defects in VHL are the cause of erythrocytosis familial type.
  Location: cytoplasmic
  Expression: Brain, Renal cell carcinoma.
• Reconstitution and Storage: Store at -20C for one year. Avoid repeated freeze/thaw cycles.
• Immunogen: Synthesized peptide derived from human pVHL
• Purification: The antibody was affinity-purified from mouse ascites by affinity-chromatography using specific immunogen.
• Concentration: 1 mg/ml
• Specificity: This antibody detects endogenous levels of human pVHL
• Application Info: IHC-P 1:200

Target Info

• Gene Symbol: VHL
• Alias Symbols: RCA1, VHL1, pVHL, HRCA1
• NCBI Gene Id: 7428
• Protein Name: Von Hippel-Lindau disease tumor suppressor (Protein G7) (pVHL)
• Uniprot ID: P40337