Xeroderma Models

  • Howard Maibach
Living reference work entry


Xeroderma pigmentosum is an autosomal recessive disorder characterized by hyperphotosensitivity and multiple cancers in association with abnormal DNA repair (Robbins et al. 1974; Satokata et al. 1992). Xeroderma pigmentosum group A (XPA) gene-deficient mice cannot repair UV-induced DNA damage and easily develop skin cancers by UV irradiation (Nakane et al. 1995; Miyauchi-Hashimoto et al. 1996, 1999). Kuwamoto et al. (2000) tested the involvement of enhanced prostaglandin E2 production in the photosensitivity in xeroderma pigmentosum group A model mice and the influence of a prostaglandin synthesis inhibitor.


Xeroderma Pigmentosum Genetic Mouse Model Prostaglandin Synthesis Inhibitor Xeroderma Pigmentosum Group Develop Skin Cancer 
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References and Further Reading

  1. De Boer J, van Steeg H, Berg RJ, de Garssen J, van Witt J, Oostrum CT, van der Beems RB, van Horst GT, de Kreijl CF, Gruijl FR, Bootsma D, Hoeijmakers JK, Weeda G (1999) Mouse model for the DNA repair/basal transcription disorder trichothiodystrophy reveals cancer predisposition. Cancer Res 59:3489–3494PubMedGoogle Scholar
  2. Kuwamoto K, Miyauchi-Hashimoto H, Tanaka K, Eguchi M, Inui T, Urade Y, Horio T (2000) Possible involvement of enhanced prostaglandin E2 production in the photosensitivity in Xeroderma pigmentosum group A model mice. J Invest Dermatol 114:241–246CrossRefPubMedGoogle Scholar
  3. Miyauchi-Hashimoto H, Tanaka K, Horio T (1996) Enhanced inflammation and immunosuppression by ultraviolet radiation in xeroderma pigmentosum group A (XPA) model. J Invest Dermatol 107:343–348CrossRefPubMedGoogle Scholar
  4. Miyauchi-Hashimoto H, Okamoto H, Tanaka K, Horio T (1999) Ultraviolet radiation-induced suppression of natural killer cell activity is enhanced in xeroderma pigmentosum group A (XPA) model mice. J Invest Dermatol 112:965–970CrossRefPubMedGoogle Scholar
  5. Nakane H, Takeuchi S, Yuba S, Saijo M, Nakatsu Y, Murai H, Nakatsuru Y, Ishikawa T, Hirota S, Kitamura Y (1995) High incidence of ultraviolet-B- or chemical-carcinogen-induced skin tumors in mice lacking the xeroderma pigmentosum group A gene. Nature 337:165–168CrossRefGoogle Scholar
  6. Robbins JH, Kraemer KH, Lutzner MA, Festoff BW, Coon HG (1974) Xeroderma pigmentosum: an inherited disease with sun sensitivity, multiple cutaneous neoplasms, and abnormal DNA repair. Ann Intern Med 80:221–248CrossRefPubMedGoogle Scholar
  7. Satokata I, Tanaka K, Okada Y (1992) Molecular basis of group A xeroderma pigmentosum: a missense mutation and two deletions located in a zinc finger consensus sequence of the XPAC gene. Hum Genet 88:603–607CrossRefPubMedGoogle Scholar
  8. Sun XZ, Harada YN, Zhang R, Cui C, Takahashi S, Fukui Y (2003) A genetic mouse model carrying the nonfunctional xeroderma pigmentosum group G gene. Congenit Anom (Kyoto) 43:133–139CrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.Department of DermatologyUC San FranciscoSan FranciscoUSA

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