SRP2084
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
Produits recommandés
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
Numéro d'accès UniProt
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)
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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
Linehan, W.M., et al.
Journal of the American Medical Association, 273, 564-570 (1995)
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
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