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Transcription profiling of Cockayne Syndrome human fibroblasts lacking functional CSB, a SWI/SNF-like ATPase, to determine the molecular defect in CS

ABSTRACT: Cockayne syndrome (CS) is an inherited neurodevelopmental disorder with progeroid features. Although the genes responsible for CS have been implicated in a variety of DNA repair- and transcription-related pathways, the nature of the molecular defect in CS remains mysterious. We sought to define this defect by expression analysis of cells lacking functional CSB, a SWI/SNF-like ATPase that is responsible for most CS cases.

ORGANISM(S): Homo sapiens

SUBMITTER: John Newman

PROVIDER: E-GEOD-3407 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress

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Cockayne syndrome group B protein (CSB) plays a general role in chromatin maintenance and remodeling.

Newman John C JC Bailey Arnold D AD Weiner Alan M AM

Proceedings of the National Academy of Sciences of the United States of America 20060613 25

Cockayne syndrome (CS) is an inherited neurodevelopmental disorder with progeroid features. Although the genes responsible for CS have been implicated in a variety of DNA repair- and transcription-related pathways, the nature of the molecular defect in CS remains mysterious. Using expression microarrays and a unique method for comparative expression analysis called L2L, we sought to define this defect in cells lacking a functional CS group B (CSB) protein, the SWI/SNF-like ATPase responsible for ...[more]

PMID: 16772382

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