Williams-Beuren syndrome-associated transcription factor TFII-I regulates osteogenic marker genes.

Abstract	Williams-Beuren syndrome (WBS), an autosomal dominant genetic disorder, is characterized by a unique cognitive profile and craniofacial defects. WBS results from a microdeletion at the chromosomal location 7q11.23 that encompasses the genes encoding the members of TFII-I family of transcription factors. Given that the haploinsufficiency for TFII-I is causative to the craniofacial phenotype in humans, we set out to analyze the effect of post-transcriptional silencing of TFII-I during BMP-2-driven osteoblast differentiation in the C2C12 cell line. Our results show that TFII-I plays an inhibitory role in regulating genes that are essential in osteogenesis and intersects with the bone-specific transcription factor Runx2 and the retinoblastoma protein, pRb. Identification of pathways regulated by TFII-I family transcription factors may begin to shed light on the molecular determinants of WBS.
Authors	Maria B Lazebnik, Maria Isabel Tussie-Luna, Philip W Hinds, Ananda L Roy
Journal	The Journal of biological chemistry (J Biol Chem) Vol. 284 Issue 52 Pg. 36234-36239 (Dec 25 2009) ISSN: 1083-351X [Electronic] United States
PMID	19880526 (Publication Type: Journal Article, Research Support, N.I.H., Extramural)
Chemical References	Antigens, Differentiation Bmp2 protein, mouse Bone Morphogenetic Protein 2 Core Binding Factor Alpha 1 Subunit GTF2I protein, human Gtf2i protein, mouse RUNX2 protein, human Retinoblastoma Protein Runx2 protein, mouse Transcription Factors, TFII
Topics	Animals Antigens, Differentiation (biosynthesis, genetics) Bone Morphogenetic Protein 2 (pharmacology) COS Cells Cell Differentiation (drug effects, genetics) Chlorocebus aethiops Chromosome Deletion Chromosomes, Human, Pair 7 (genetics, metabolism) Core Binding Factor Alpha 1 Subunit (biosynthesis, genetics) Humans Mice NIH 3T3 Cells Osteoblasts (metabolism) Osteogenesis RNA Interference Retinoblastoma Protein (biosynthesis, genetics) Transcription Factors, TFII (genetics, metabolism) Williams Syndrome (genetics, metabolism)

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