Applications of the Diazacholesterol Animal Model of Ichthyosis
Although scaling skin diseases traditionally have been considered disorders of protein metabolism, i.e., keratinization, there is little support for this hypothesis. Instead, the expanding list of inherited and acquired disorders that are associated with primary lipid abnormalities (Table 1, Fig. 1), implies that it is stratum corneum lipids which regulate stratum corneum cohesion and desquamation (reviewed in [4, 16]). Whereas relevant examples of inherited scaling dermatoses that are associated with abnormal lipid metabolism include recessive x-linked ichthyosis (RCLI), Refsum’s disease; multiple sulfatase deficiency, and neutral lipid storage disease, examples of acquired disorders include ichthyosis occurring following administration of hypocholesterolemic agents (reviewed in ). Essential fatty acid deficiency (EFAD), both in rodents and in man, represents an additional scaling disorder where stratum corneum barrier function and scaling have been attributed to separate abnormalities in lipid metabolism .
KeywordsStratum Corneum Free Sterol Hairless Mouse Essential Fatty Acid Deficiency Stratum Corneum Lipid
Unable to display preview. Download preview PDF.
- 3.Chung J-C, Law MYL, Elliott ST, Elias PM (1984) Diazacholesterolinduced ichthyosis in the hairless mouse: Assay for comparative potency of topical retinoids. Arch Dermatol 120: 342–347Google Scholar
- 5.Elias PM, Brown (1978) The mammalian cutaneous permeability barrier: defective barrier function in essential fatty acid deficiency correlates with abnormal intercellular lipid composition. Lab Invest 39: 574–583Google Scholar
- 11.Lampe MA, Burlingame AL, Whitney J, Williams ML, Brown BE, Roitman E, Elias PM (1983) Human stratum corneum lipids: Characterization and regional variations. J Lipid Res 24: 120–130Google Scholar
- 12.Mezick JA, Chabria MC, Thorne EG, Capetola RJ (1982) Topical and oral (P.O.) effects of 13-cis-retinoic acid (13-cis-RA) and all-trans retinoic acid ( RA) on horn-filled utriculi size in the Rhino mouse. Clin Res 30: 598AGoogle Scholar
- 17.William ML, Elias PM (1981) Stratum corneum lipids in disorders of cornification I. Increased cholesterol sulfate content of stratum corneum in recessive x-linked ichthyosis. J Clin Invest 68: 1404–1410Google Scholar
- 18.Williams ML, Elias PM (1982) n-Alkanes in normal and pathological human scale. Biochem Biophys Res Comm 107: 322–328Google Scholar