STANDARD_NAME	NEWMAN_ERCC6_TARGETS_UP
SYSTEMATIC_NAME	M5459
COLLECTION	C2:CGP
MSIGDB_URL	https://www.gsea-msigdb.org/gsea/msigdb/human/geneset/NEWMAN_ERCC6_TARGETS_UP
NAMESPACE	AFFY_HG_U133
DESCRIPTION_BRIEF	Genes up-regulated in Cockayne syndrome fibroblasts rescued by expression of ERCC6 [GeneID=2074] off a plasmid vector.
DESCRIPTION_FULL	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 most cases of CS. Remarkably, many of the genes regulated by CSB are also affected by inhibitors of histone deacetylase and DNA methylation, as well as by defects in poly(ADP-ribose)-polymerase function and RNA polymerase II elongation. Moreover, consistent with these microarray expression data, CSB-null cells are sensitive to inhibitors of histone deacetylase or poly(ADP-ribose)-polymerase. Our data indicate a general role for CSB protein in maintenance and remodeling of chromatin structure and suggest that CS is a disease of transcriptional deregulation caused by misexpression of growth-suppressive, inflammatory, and proapoptotic pathways.
PMID	16772382
GEOID	GSE3407
AUTHORS	Newman JC,Bailey AD,Weiner AM
CONTRIBUTOR	Leona Saunders
CONTRIBUTOR_ORG	MSigDB Team
EXACT_SOURCE	Table 3S
FILTERED_BY_SIMILARITY	
EXTERNAL_NAMES_FOR_SIMILAR_TERMS	
EXTERNAL_DETAILS_URL	
SOURCE_MEMBERS	200704_at,202345_s_at,202859_x_at,204244_s_at,204285_s_at,204286_s_at,204971_at,205081_at,205542_at,206025_s_at,206026_s_at,206837_at,208650_s_at,208920_at,208921_s_at,209774_x_at,209921_at,209993_at,209994_s_at,210663_s_at,211506_s_at,213652_at,217388_s_at,217678_at,218237_s_at,218723_s_at,219863_at,224480_s_at,226757_at,227012_at,227230_s_at,228098_s_at,228707_at,236277_at,243951_at
GENE_SYMBOLS	LITAF,FABP5,CXCL8,DBF4,PMAIP1,PMAIP1,CSTA,CRIP1,STEAP1,TNFAIP6,TNFAIP6,ALX1,CD24P2,SRI,SRI,CXCL2,SLC7A11,ABCB1,ABCB1,KYNU,CXCL8,PCSK5,KYNU,SLC7A11,SLC38A1,RGCC,HERC5,GPAT3,IFIT2,SLC25A40,CRACD,MYLIP,CLDN23,,ABCB1
FOUNDER_NAMES