Epigenetic Silencing of β-Spectrin, a TGF-β Signaling/Scaffolding Protein in a Human Cancer Stem Cell Disorder: BECKWITH-WIEDEMANN SYNDROME

• Published in: The Journal of biological chemistry Vol. 285; no. 46; pp. 36112 - 36120
• Main Authors: Yao, Zhi-Xing, Jogunoori, Wilma, Choufani, Sanaa, Rashid, Asif, Blake, Tiffany, Yao, Wenguo, Kreishman, Peter, Amin, Rupen, Sidawy, Anton A, Evans, Stephen R.T, Finegold, Milton, Reddy, E. Premkumar, Mishra, Bibhuti, Weksberg, Rosanna, Kumar, Rakesh, Mishra, Lopa
• Format: Journal Article
• Language: English
• Published: United States American Society for Biochemistry and Molecular Biology 12.11.2010
• Subjects: Animals; Azacitidine - analogs & derivatives; Azacitidine - pharmacology; Base Sequence; Beckwith-Wiedemann Syndrome - genetics; Blotting, Western; Decitabine; DNA Methylation - drug effects; Enzyme Inhibitors - pharmacology; Epigenesis, Genetic; Epigenomics; Gene Expression Profiling; Hep G2 Cells; Heterozygote; Humans; Insulin-Like Growth Factor II - genetics; Insulin-Like Growth Factor II - metabolism; Mice; Mice, Knockout; Molecular Bases of Disease; Neoplastic Stem Cells - metabolism; Oligonucleotide Array Sequence Analysis; Phenotype; Promoter Regions, Genetic - genetics; Spectrin - genetics; Spectrin - metabolism; Tumor Cells, Cultured

Hereditary cancer syndromes provide powerful insights into dysfunctional signaling pathways that lead to sporadic cancers. Beckwith-Wiedemann syndrome (BWS) is a hereditary human cancer stem cell syndrome currently linked to deregulated imprinting at chromosome 11p15 and uniparental disomy. However,...