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Key Documents

SRP2084

Sigma-Aldrich

VHL human

recombinant, expressed in insect cells, ≥70% (SDS-PAGE)

Synonyme(s) :

HRCA1, RCA1, VHL1

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About This Item

Code UNSPSC :
12352200
Nomenclature NACRES :
NA.26

Source biologique

human

Produit recombinant

expressed in insect cells

Pureté

≥70% (SDS-PAGE)

Forme

frozen liquid

Poids mol.

~26 kDa

Conditionnement

pkg of 5 μg

Conditions de stockage

avoid repeated freeze/thaw cycles

Concentration

650 μg/mL

Couleur

clear colorless

Numéro d'accès NCBI

NM_000551

Numéro d'accès UniProt

P40337

Conditions d'expédition

dry ice

Température de stockage

−70°C

Informations sur le gène

human ... VHL(7428)

Actions biochimiques/physiologiques

von Hippel-Lindau (VHL) disease is a hereditary cancer with a predilection for the central nervous system and retina. The von Hippel-Lindau tumor suppressor gene is mutated in families with von Hippel-Lindau disease and encodes a protein (VHL) of 213 amino acids with an acidic pentapeptide motif in the N-terminus. Mutations in the VHL gene result in constitutive expression of many hypoxia-induced genes, at least in part because of increases in the cellular level of hypoxia-inducible transcription factor HIF-1a. VHL protein binds to elongin B, elongin C, and Cul2 to form a stable complex that targets hypoxia inducible factors (HIFs) for degradation and transcriptional regulation. In addition, VHL protein has also been shown to interact with specific protein kinase C isoforms, histone deacetylases and HIF-1 inhibitor (HIF-1).

Forme physique

Clear and colorless frozen liquid solution

Notes préparatoires

Use a manual defrost freezer and avoid repeated freeze-thaw cycles. While working, please keep sample on ice.

Code de la classe de stockage

10 - Combustible liquids

Classe de danger pour l'eau (WGK)

WGK 1

Point d'éclair (°F)

Not applicable

Point d'éclair (°C)

Not applicable

Certificats d'analyse (COA)

Recherchez un Certificats d'analyse (COA) en saisissant le numéro de lot du produit. Les numéros de lot figurent sur l'étiquette du produit après les mots "Lot" ou "Batch".

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Consulter la Bibliothèque de documents

Linehan, W.M., et al.
Journal of the American Medical Association, 273, 564-570 (1995)
A D Singh et al.
Survey of ophthalmology, 46(2), 117-142 (2001-10-02)
In recent years advances have been made in the clinical and genetic aspects of von Hippel-Lindau disease (VHL). Retinal capillary hemangioma is the most common manifestation of VHL disease and, therefore, ophthalmologists are frequently involved in the care of patients
F Latif et al.
Science (New York, N.Y.), 260(5112), 1317-1320 (1993-05-28)
A gene discovered by positional cloning has been identified as the von Hippel-Lindau (VHL) disease tumor suppressor gene. A restriction fragment encompassing the gene showed rearrangements in 28 of 221 VHL kindreds. Eighteen of these rearrangements were due to deletions
E Hasanov et al.
Oncogene, 36(24), 3450-3463 (2017-01-24)
The hypoxia-regulated tumor-suppressor von Hippel-Lindau (VHL) is an E3 ligase that recognizes its substrates as part of an oxygen-dependent prolyl hydroxylase (PHD) reaction, with hypoxia-inducible factor α (HIFα) being its most notable substrate. Here we report that VHL has an
Jung Jin Kim et al.
Genes & development, 29(21), 2244-2257 (2015-11-08)
The von Hippel-Lindau tumor suppressor pVHL is an E3 ligase that targets hypoxia-inducible factors (HIFs). Mutation of VHL results in HIF up-regulation and contributes to processes related to tumor progression such as invasion, metastasis, and angiogenesis. However, very little is

Articles

Oncogenes and Tumor Suppressors Reprogram Metabolism

Sigma-Aldrich presents an article about how proliferating cells require the biosynthesis of structural components for biomass production and for genomic replication.

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