Phenotypes associated with this allele
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Xpctm1Ecf mutation
(1 available);
any
Xpc mutation
(34 available)
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cellular
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• murine embryonic fibroblasts are more sensitive to UV radiation than wild-type cells
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• increased sensitivity to oxidative stress
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Xpctm1Ecf mutation
(1 available);
any
Xpc mutation
(34 available)
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Lungs of Xpctm1Ecf/Xpctm1Ecf mice show a range of lesions
neoplasm
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• lung tumors are seen as early as 6 months of age and by 16-17 months of age, all mutants develop tumors
• incidence and multiplicity of lung tumors increases with age
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• most lung tumors are classified as adenomas, with only a few progressing to malignant carcinomas
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• a few lung tumors progress to malignant carcinomas
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respiratory system
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• lung tumors are seen as early as 6 months of age and by 16-17 months of age, all mutants develop tumors
• incidence and multiplicity of lung tumors increases with age
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• most lung tumors are classified as adenomas, with only a few progressing to malignant carcinomas
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• a few lung tumors progress to malignant carcinomas
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Xpctm1Ecf mutation
(1 available);
any
Xpc mutation
(34 available)
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integument
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• cyclobutane pyrimidine dimers are observed in hair follicles after treatment with a total UV dose of 1.0 kJ/m2
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• basal cells exhibit hypoplasia after a total UV dose of 1.0 kJ/m2 despite the dose being much below the minimal erythema dose
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• cyclobutane pyrimidine dimers are observed in cells within the dermis at a reduced level (4.7% compared to 12% in wild-type mice) after treatment with a total UV dose of 1.0 kJ/m2
• pyridine pirimidone photoproducts accumulate in about 1.8% of epidermal basal cells after treatment with a total UV dose of 1.0 kJ/m2 and no increase in photoproducts with increased exposure
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Allelic Composition |
Xpctm1Ecf/Xpc+
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Genetic Background |
involves: 129 * SKH1 |
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Xpctm1Ecf mutation
(1 available);
any
Xpc mutation
(34 available)
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integument
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• basal cells exhibit reduced levels of cyclobutane pyrimidine dimers (8.7% compared to 12% in wild-type mice) after a total UV dose of 1.0 kJ/m2
• however, no pyridine pirimidone photoproducts accumulate
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ercc3tm1.1Gima mutation
(0 available);
any
Ercc3 mutation
(31 available)
Xpctm1Ecf mutation
(1 available);
any
Xpc mutation
(34 available)
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cellular
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• UV-treated dermal fibroblasts exhibit reduced survival compared with similarly treated wild-type cells
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mortality/aging
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• at 24 months of age, only 28% of mutants are alive compared to 43% of wild-type
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neoplasm
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• some mutants exhibit malignant adenocarcinoma with metastasis to adjacent lymph nodes
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• 100% of mutants develop lung tumors by 24 months of age; display a full range of lung lesions, ranging from mild atypia and hyperplasia to malignant adenocarcinoma with metastasis to adjacent lymph nodes
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• majority of mutants develop lung adenocarcinomas coincident with adenomas
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respiratory system
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• 100% of mutants develop lung tumors by 24 months of age; display a full range of lung lesions, ranging from mild atypia and hyperplasia to malignant adenocarcinoma with metastasis to adjacent lymph nodes
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• majority of mutants develop lung adenocarcinomas coincident with adenomas
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Apex1tm1Ecf mutation
(0 available);
any
Apex1 mutation
(16 available)
Xpctm1Ecf mutation
(1 available);
any
Xpc mutation
(34 available)
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neoplasm
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• 16% developed microscopic tumors
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cellular
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• increased sensitivity to oxidative stress
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mortality/aging
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• 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
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growth/size/body
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• develop progressive cachexia in the third week of life
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homeostasis/metabolism
cellular
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• MEFs are more UV-sensitive
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• analysis of UV-induced repair synthesis and RNA synthesis recovery show complete inactivation of nucleotide excision repair (NER)
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ercc8tm1Jhjh mutation
(0 available);
any
Ercc8 mutation
(31 available)
Xpctm1Ecf mutation
(1 available);
any
Xpc mutation
(34 available)
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mortality/aging
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• animals within a month of birth
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growth/size/body
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ercc8tm1Jhjh mutation
(0 available);
any
Ercc8 mutation
(31 available)
Xpctm1Ecf mutation
(1 available);
any
Xpc mutation
(34 available)
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neoplasm
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• mutants develop skin tumors after chronic exposure to UVB radiation at an accelerated rate compared to single Ercc8 homozygotes
• mutants exposed to UVB radiation also develop eye tumors, most being hemangiosarcomas
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cellular
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• fewer epidermal cells accumulate in S-phase and in G2-phase after treatment with 500J/m2 of UV radiation than in mice carrying Xpctm1Ecf alone
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