Ichthyosis vulgaris is a heterogeneous autosomal skin disease characterized by dry, scaly skin, mild hyperkeratosis, and a decreased or absent granular layer that either lacks or contains morphologically abnormal, keratohyalin granula (Anton-Lamprecht and Hofbauer 1972). Both the skin of ichthyosis vulgaris patients and keratinocytes cultured from affected individuals exhibit reduced or absent profilaggrin mRNA and protein levels (Sybert et al. 1985). The symptoms and the genetics of the ichthyotic (ic/ic) mouse were described by Spearman (1960), Green et al. (1974), Jensen and Esterly (1977), and Holbrook (1989). Presland et al. (2000) demonstrated loss of normal profilaggrin and filaggrin in flaky tail (ft/ft) mice and proposed this as an animal model for the filaggrin-deficient skin disease ichthyosis vulgaris.
KeywordsHairless Mouse Topical Retinoid Comparative Potency Single Gene Model Scaly Skin
References and Further Reading
- Knox WE, Lister-Rosenoer LM (1998) Infantile ichthyosis in rats: a new model of hyperkeratotic skin disease. J Hered 69:391–394Google Scholar
- Shultz LD, Lyons BL, Burzenski LM, Gott B, Samuels R, Schweitzer PA, Dreger C, Herrmann H, Kalscheuer V, Olins AL, Olins DE, Sperling K, Hoffmann K (2003) Mutations at the mouse ichthyosis locus are within the lamin B receptor gene: a single gene model for human Pelger-Huet anomaly. Hum Mol Genet 12:61–69CrossRefPubMedGoogle Scholar
- Spearman RJ (1960) The skin abnormality of “ichthyosis”, a mutant of the house mouse. J Embryol Exp Morphol 8:387–395Google Scholar