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neoplastic disease that affects approximately 1/36000–45,500 live births [1,2]. VHL disease encompasses various types of tumors, including retinal or central nervous system hemangioblastoma (RH or CNSH), clear cell renal cell carcinoma (RCC), pheochromocytoma (PHE), multiple pancreatic cysts or...
A–CThe protein expression of VHL in U373-shNC/shFBXO22, LN229-shNC/shFBXO22, and U87-Vector/FBXO22 cells was detected.D,EVHL mRNA expression in FBXO22 knockdown cells and FBXO22 overexpression cells was assessed by qRT-PCR assay.F,GAfter treatment with CHX, VHL protein expression was...
Hypoxia-inducible factor-1α (HIF-1α) plays central roles in the hypoxia response. It is highly expressed in multiple cancers, but not always correlated with hypoxia. Mutation of the von Hippel–Lindau (VHL) gene, which encodes an E3 ligase, contributes to the constructive activation of HIF...
PubMed CentralGoogle Scholar Allred, D. C., Brown, P. & Medina, D. The origins of estrogen receptor alpha-positive and estrogen receptor alpha-negative human breast cancer.Breast Cancer Res.6, 240–245 (2004). CASPubMedPubMed CentralGoogle Scholar ...
in VHL patients are hemangioblastomas of the retina or optic nerve head. A hemangioblastoma is any case of angiomatosis, a proliferation of vascular endothelium, within the central nervous system. Retinal capillary hemangioblastomas are found in up to 70 % of VHL patients by 60 years of...
J. Oxygen sensing by metazoans: the central role of the HIF hydroxylase pathway. Mol. Cell 30, 393–402 (2008). 7. Majmundar, A. J., Wong, W. J. & Simon, M. C. Hypoxia-inducible factors and the response to hypoxic stress. Mol. Cell 40, 294–309 (2010). 8. Kamura, T. et...
HNF-4α was found to be a central node in the network in RCC4 (Supplementary Fig. 7a) and 786-O (Supplementary Fig. 7b). In addition, several HNF-4α target genes were found in these VHL alone-regulated genes (Supplementary Data 15). Thus, we extrapolated that HNF-4α might be inv...
Id2 mediates tumor initiation, proliferation, and angiogenesis in Rb mutant mice. Mol. Cell. Biol. 25, 3563–3574 (2005) CAS PubMed PubMed Central Google Scholar Niola, F. et al. Mesenchymal high-grade glioma is maintained by the ID–RAP1 axis. J. Clin. Invest. 123, 405–417 (...
Chemical strategies to using small molecules to stimulate hypoxia inducible factors (HIFs) activity and trigger a hypoxic response under normoxic conditions, such as iron chelators and inhibitors of prolyl hydroxylase domain (PHD) enzymes, have broad-spe