Stock B6;129-Xpa<tm1Hvs>Trp53<tm1holl>Tg(pUR288)1Vij/H

FESA Number

6596

Strain Name

B6;129-Xpa<tm1Hvs>Trp53<tm1holl>Tg(pUR288)1Vij/H

Mutation Strain Type

Attribution

Gene/Allele Information

Allele Name Allele MGI ID Gene Name Gene MGI ID Chromosome
Trp53<tm1Holl> MGI:2157167 Trp53 MGI:98834 11
Xpa<tm1Hvs> MGI:1857939 Xpa MGI:99135 4
Tg(pUR288)1Vij MGI:3606518 Tg(pUR288)1Vij MGI:3606513 11

Severity Limit

Mild

Phenotypic Description

Mice that are homozygous for the targeted Trp53 mutation (i.e. Hupki) are healthy, fertile and do not display any gross abnormalities. The expression and functional activity of the chimeric Trp53 gene in homozygous Hupki mice is normal. Homozygous Hupki mice that are also homozygous for the targeted Xpa knockout mutation (i.e. Xpa-Null) are viable and fertile and do not undergo spontaneous tumour development. (However, the stock was not maintained by homozygous matings, but by mating Xpa<tm1Hvs>/+ to +/+). Xpa is required for nucleotide excision repair (NER), therefore Xpa-Null mice and cells are completely deficient in NER. Hupki:Xpa-Null mice and embryo fibroblasts are highly sensitive to carcinogens that generate DNA damage normally repaired by NER (e.g. bulky DNA adducts) compared with Hupki:Xpa-wild-type controls (unpublished data).

Orphanet Disorders

OrphaNet ID Name Url
276 249 Xeroderma pigmentosum complementation group A http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=276249

Publications

Displaying 1 - 10 of 81 Stock Publications
Pubmed ID Authors Title Journal
17172844 Feng L Ser46 phosphorylation regulates p53-dependent apoptosis and replicative senescence. Cell Cycle (2 006) 5:2812-9
9661901 Bol SA Elevated frequencies of benzo(a)pyrene-induced Hprt mutations in internal tissue of XPA-deficient mice. Cancer Res (1 998) 58:2850-6
10862569 Gulezian D Use of transgenic animals for carcinogenicity testing: considerations and implications for risk assessment. Toxicol Pathol (2 000) 28:482-99
21151170 Melis JP Genotoxic exposure: novel cause of selection for a functional ?N-p53 isoform. Oncogene (2 011) 30:1764-72
21715664 van Kesteren PC Deregulation of cancer-related pathways in primary hepatocytes derived from DNA repair-deficient Xpa-/-p53+/- mice upon exposure to benzo[a]pyrene. Toxicol Sci (2 011) 123:123-32
6315402 Rüther U Easy identification of cDNA clones. EMBO J (1 983) 2:1791-4
21402718 Feng Z Regulation of female reproduction by p53 and its family members. FASEB J (2 011) 25:2245-55
11442761 Boonstra A Differential ultraviolet-B-induced immunomodulation in XPA, XPC, and CSB DNA repair-deficient mice. J Invest Dermatol (2 001) 117:141-6
15289314 Hoogervorst EM p53 heterozygosity results in an increased 2-acetylaminofluorene-induced urinary bladder but not liver tumor response in DNA repair-deficient Xpa mice. Cancer Res (2 004) 64:5118-26
23153559 Melis JP Detection of genotoxic and non-genotoxic carcinogens in Xpc(-/-)p53(+/-) mice. Toxicol Appl Pharmacol (2 013) 266:289-97

Pages

Orphanet Categories

Rare developmental defect during embryogenesis
Rare neurologic disease
Rare skin disease
Rare oncologic disease

Genetic Status

GMO

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