Expression of the excision repair gene, ERCC3 (excision repair cross-complementing), during mouse development.
Expression of the human ERCC3 (excision repair cross-complementing) gene in cells from patients with xeroderma pigmentosum (XP) group B (XP-B) corrects the defect in repair of UV light-induced DNA damage. XP-B is one of three groups of XP which exhibit the clinical symptoms of both XP and Cockayne's Syndrome (CS). CS and XP-B/CS patients develop severe neurological dysfunction during development. In order to explore the link between the defective gene and the neurological deficits in XP/CS, we have studied the expression of ERCC3 mRNA in developing mice by in situ hybridisation. ERCC3 was found to be ubiquitously expressed in cells from all regions and all developmental stages, from 9 day post-coitum embryo, to 15 day post-natal brain. In post-natal brain, regional differences in expression correlated with cell density and there was no evidence of cell specific or developmental alterations in levels of expression. These results indicate that the constitutively expressed gene does not perform a discrete developmental function. The neurological defects apparent in XP-B are likely to arise pleiotypically from the participation of ERCC3 in interactions with other elements involved in particular aspects of neurodevelopmental control. These results emphasise the developmental importance of genes whose primary functions are apparently unconnected with development.
Hubank
M
M
Trafford Centre for Medical Research, University of Sussex, Falmer, Brighton, UK.
Mayne
L
L
D000818:Animals; D001921:Brain / Q000254:growth & development* / Q000378:metabolism; D003057:Cockayne Syndrome / Q000378:metabolism; D004249:DNA Damage; D004265:DNA Helicases; D015342:DNA Probes; D004260:DNA Repair; D004268:DNA-Binding Proteins / Q000235:genetics*; D015870:Gene Expression / Q000502:physiology*; D017403:In Situ Hybridization; D051379:Mice; D012333:RNA, Messenger / Q000096:biosynthesis
D015342:DNA Probes; D004268:DNA-Binding Proteins; D012333:RNA, Messenger; C072022:XPBC-ERCC-3 protein; D004265:DNA Helicases