CECR2, a protein involved in neurulation, forms a novel chromatin remodeling complex with SNF2L
Open Access
- 7 January 2005
- journal article
- research article
- Published by Oxford University Press (OUP) in Human Molecular Genetics
- Vol. 14 (4) , 513-524
- https://doi.org/10.1093/hmg/ddi048
Abstract
Chromatin remodeling complexes play critical roles in development. Here we describe a transcription factor, CECR2, which is involved in neurulation and chromatin remodeling. CECR2 shows complex alternative splicing, but all variants contain DDT and bromodomain motifs. A mutant mouse line was generated from an embryonic stem cell line containing a genetrap within Cecr2. Reporter gene expression demonstrated Cecr2 expression to be predominantly neural in the embryo. Mice homozygous for the Cecr2 genetrap-induced mutation show a high penetrance of the neural tube defect exencephaly, the human equivalent of anencephaly, in a strain-dependent fashion. Biochemical isolation of CECR2 revealed the presence of this protein as a component of a novel heterodimeric complex termed CECR2-containing remodeling factor (CERF). CERF comprises CECR2 and the ATP-dependent chromatin remodeler SNF2L, a mammalian ISWI ortholog expressed predominantly in the central nervous system. CERF is capable of remodeling chromatin in vitro and displays an ATP hydrolyzing activity that is stimulated by nucleosomes. Together, these data identify a novel chromatin remodeling complex with a critical role in neurulation.Keywords
This publication has 52 references indexed in Scilit:
- Growth retardation and premature aging phenotypes in mice with disruption of the SNF2-like gene, PASGGenes & Development, 2004
- Chromatin remodeling by ATP‐dependent molecular machinesBioEssays, 2003
- Identification of acquired somatic mutations in the gene encoding chromatin-remodeling factor ATRX in the α-thalassemia myelodysplasia syndrome (ATMDS)Nature Genetics, 2003
- Syndromes of disordered chromatin remodelingClinical Genetics, 2003
- Biological functions of the ISWI chromatin remodeling complex NURFGenes & Development, 2002
- Human diseases with underlying defects in chromatin structure and modificationHuman Molecular Genetics, 2001
- What does ‘chromatin remodeling’ mean?Trends in Biochemical Sciences, 2000
- The ISWI Chromatin-Remodeling Protein Is Required for Gene Expression and the Maintenance of Higher Order Chromatin Structure In VivoMolecular Cell, 2000
- ATRX encodes a novel member of the SNF2 family of proteins: mutations point to a common mechanism underlying the ATR-X syndromeHuman Molecular Genetics, 1996
- ISWI, a member of the SWl2/SNF2 ATPase family, encodes the 140 kDa subunit of the nucleosome remodeling factorCell, 1995