A Drosophila model for xeroderma pigmentosum and Cockayne's syndrome: haywire encodes the fly homolog of ERCC3, a human excision repair gene.

Abstract:

:The haywire gene of Drosophila encodes a protein with 66% identity to the product of the human ERCC3 gene, associated with xeroderma pigmentosum B (XP-B) and Cockayne's syndrome (CS). XP is a human autosomal recessive disease characterized by extreme sensitivity to ultraviolet irradiation and marked susceptibility to skin cancer. In addition, XP and CS patients often exhibit a variety of defects, ranging from central nervous system disorders to hypogonadism. Phenotypes of haywire mutants mimic some of the effects of XP. Many haywire alleles are recessive lethal, viable alleles cause ultraviolet sensitivity, and files expressing marginal levels of haywire display motor defects and reduced life span. Progeny of females carrying a maternal effect allele show central nervous system defects.

journal_name

Cell

journal_title

Cell

authors

Mounkes LC,Jones RS,Liang BC,Gelbart W,Fuller MT

doi

10.1016/0092-8674(92)90389-t

subject

Has Abstract

pub_date

1992-12-11 00:00:00

pages

925-37

issue

6

eissn

0092-8674

issn

1097-4172

pii

0092-8674(92)90389-T

journal_volume

71

pub_type

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