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Exploring the Role of Defective Fibronectin Matrix Assembly in the VHL-Associated CNS Hemangioblastoma.

Publication Type : Journal Article

Thematic Areas : Medical Sciences, Nanosciences and Molecular Medicine

Publisher : Drug Metab Pers Ther

Source : Drug Metab Pers Ther

Campus : Kochi

School : Center for Nanosciences, School of Medicine

Center : Amrita Center for Nanosciences and Molecular Medicine Move, Nanosciences

Department : Biochemistry, Nanosciences and Molecular Medicine, Neurosurgery

Verified : No

Year : 2018

Abstract : Background: Central nervous system (CNS) hemangioblastoma (HB) is the most common tumor in the von Hippel Lindau (VHL) disorder, the hereditary tumor syndrome caused by the biallelic mutations of the VHL gene. The disrupted VHL and Elongin protein interaction on hypoxia-inducible factor-1α (HIF-1α) induces a set of hypoxia-inducible genes, resulting in an unchecked endothelial cell proliferation that then leads to hemangioblastoma formation. However, recent studies have shown that disruptive germline mutations of VHL need not result in hemangioblastoma, though it can cause other manifestations of the VHL syndrome. Similarly, sporadic hemangioblastoma can occur rarely without a somatic biallelic VHL mutation. The VHL protein was earlier found to be associated with the deposition of matrix fibronectin (FN) protein in the renal extracellular matrix.

Cite this Research Publication : N. Vikkath, Ariyannur, P., Krishnakumar N. Menon, Mr, B., and Pillai, A., “Exploring the Role of Defective Fibronectin Matrix Assembly in the VHL-Associated CNS Hemangioblastoma.”, Drug Metab Pers Ther, vol. 33, no. 3, pp. 127-134, 2018.

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