Roles of the sister chromatid cohesion apparatus in gene expression, development, and human syndromes

被引:118
作者
Dorsett, Dale [1 ]
机构
[1] St Louis Univ, Sch Med, Edward A Doisy Dept Biochem & Mol Biol, St Louis, MO 63104 USA
关键词
DE-LANGE-SYNDROME; BRACHMANN-DELANGE SYNDROME; XENOPUS EGG EXTRACTS; ROBERTS-SYNDROME; FISSION YEAST; SACCHAROMYCES-CEREVISIAE; SC PHOCOMELIA; NIPPED-B; SMC PROTEINS; CUT GENE;
D O I
10.1007/s00412-006-0072-6
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
The sister chromatid cohesion apparatus mediates physical pairing of duplicated chromosomes. This pairing is essential for appropriate distribution of chromosomes into the daughter cells upon cell division. Recent evidence shows that the cohesion apparatus, which is a significant structural component of chromosomes during interphase, also affects gene expression and development. The Cornelia de Lange (CdLS) and Roberts/SC phocomelia (RBS/SC) genetic syndromes in humans are caused by mutations affecting components of the cohesion apparatus. Studies in Drosophila suggest that effects on gene expression are most likely responsible for developmental alterations in CdLS. Effects on chromatid cohesion are apparent in RBS/SC syndrome, but data from yeast and Drosophila point to the likelihood that changes in expression of genes located in heterochromatin could contribute to the developmental deficits.
引用
收藏
页码:1 / 13
页数:13
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