Mouse Genome Informatics
cx
    Ercc6tm1Gvh/Ercc6tm1Gvh
Xpatm1Hvs/Xpatm1Hvs

involves: 129P2/OlaHsd * C57BL/6J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       

Ercc6tm1Gvh/Ercc6tm1Gvh Xpatm1Hvs/Xpatm1Hvs mice display postnatal growth retardation

mortality/aging
• number of double mutant pups is about 3-fold below the expected numbers, however normal numbers are seen at E18.5, indicating some lethality during or shortly after birth
• die before P22

growth/size
• develop progressive cachexia in the third week of life

homeostasis/metabolism
• analysis of UV-induced repair synthesis and RNA synthesis recovery show complete inactivation of nucleotide excision repair (NER)

cellular
• analysis of UV-induced repair synthesis and RNA synthesis recovery show complete inactivation of nucleotide excision repair (NER)
• MEFs are more UV-sensitive than single Xpa mutants

Mouse Models of Human Disease
OMIM IDRef(s)
Cockayne Syndrome B; CSB 133540 J:122013