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The quantification of free sphingosine in the stratum corneum of patients with hereditary ichthyosis
D.G. PAIGE,
N. MORSE-FISHER,
J.I. HARPER,
D.G. PAIGE
Department of Dermatology, Hospital for Sick Children, Great Ormond Street, London, U.K.
Search for more papers by this authorN. MORSE-FISHER
Efamol Research Inc., Kentville, Nova Scotia, Canada
Search for more papers by this authorCorresponding Author
J.I. HARPER
Department of Dermatology, Hospital for Sick Children, Great Ormond Street, London, U.K.
Dr J.I.Harper, Dermatology Department, Hospital for Sick Children, Great Ormond Street, London WCIN 3JH, U.K.Search for more papers by this authorD.G. PAIGE,
N. MORSE-FISHER,
J.I. HARPER,
D.G. PAIGE
Department of Dermatology, Hospital for Sick Children, Great Ormond Street, London, U.K.
Search for more papers by this authorN. MORSE-FISHER
Efamol Research Inc., Kentville, Nova Scotia, Canada
Search for more papers by this authorCorresponding Author
J.I. HARPER
Department of Dermatology, Hospital for Sick Children, Great Ormond Street, London, U.K.
Dr J.I.Harper, Dermatology Department, Hospital for Sick Children, Great Ormond Street, London WCIN 3JH, U.K.Search for more papers by this authorSummary
Sphingosine is a long-chain base which provides the back-bone of all sphingolipid molecules. Free sphingosine is found in normal epidermis, especially in the stratum corneum. As a free molecule it may modify epidermal cell proliferation and differentiation through its inhibition of protein kinase C. Using a thin-layer chromatography technique we have demonstrated in vitro that the erythrodermic ichthyoses show significantly lower levels of stratum corneum sphingosine than the non-erythrodermic types. The exact in vivo significance of this finding is unclear, but free sphingosine may have an important role in determining the inflammatory component of the hereditary ichthyoses.
References
- 1 Gray GM, White RJ. Glycosphingolipids and ceramides in human and pig epidermis. J Invest Dermatol 1978; 70: 336–41.
- 2 Wertz PW, Miethke MC, Long SA et al. The composition of the ceramides from human stratum corneum and from comedones. J Invest Dermatol 1985; 84: 410–12.
- 3 Imokawa G, Abe A, Jin K et al. Decreased level of ceramides in stratum corneum of atopic dermatitis: an aetiologic factor in atopic dry skin.' J Invest Dermatol 1991; 96: 523–6.
- 4 Yardley HJ. Epidermal lipids. In: Biochemistry and Physiology of the Skin ( LA. Goldsmith ed). Oxford : Oxford University Press, 1983; 363–81.
- 5 Elias PM. Epidermal lipids, barrier function and desquamation. J Invest Dermatol 1983; 80 (Suppl.): 44–49s.
- 6 Wertz PW, Swartzendruber DC, Kitko DJ et al. The role of the corneocyte lipid envelopes in cohesion of the stratum corneum. J Invest Dermatol 1989; 93: 169–72.
- 7 Downing DT, Stewart ME, Wertz PW et al. Skin lipids: an update. J Invest Dermatol 1987: 88 (Suppl.): 2–6s.
- 8 Wertz PW, Downing DT. Free sphingosine in porcine epidermis. Biochim Biophys Acta 1989; 1002: 213–17.
- 9 Wertz PW, Downing DT. Free sphingosine in human epidermis. J Invest Dermatol 1990; 94: 159–61.
- 10 Kobayashi T, Mitsuo K, Goto I. Free sphingoid bases in normal murine tissues. Eur J Biochem 1988; 172: 747–52.
- 11 Merrill AH Jr, Wang E, Mullins RE et al. Quantitation of free sphingosine in liver by high-performance liquid chromatography. Anal Biochem 1988; 171: 373–81.
- 12 Kobayashi T, Shinnoh N, Goto I. Metabolism of free sphingoid bases in murine tissues and in cultured human fibroblasts. Eur J Biochem 1989; 186: 493–9.
- 13 Wertz PW, Downing DT. Ceramidase activity in porcine epidermis. FEBS Lett 1990; 268: 110–12.
- 14 Hannun YA, Bell RM. Functions of sphingolipids and sphingolipid breakdown products in cellular regulation. Science 1989; 243: 500–7.
- 15 Jefferson AB, Schulman H. Sphingosine inhibits calmodulin- dependent enzymes. J Biol Chem 1988; 263: 15241–4.
- 16 Merrill AH Jr, Stevens VL. Modulation of protein kinase C and diverse cell functions by sphingosine—a pharmacologically interesting compound linking sphingolipids and signal transduction. Biochim Biophys Acta 1989; 1010: 131–9.
- 17 Hannun YA, Loomis CR, Merrill AH Jr, Bell RM. Sphingosine inhibition of protein kinase C activity and of phorbol dibutyrate binding in vitro and in human platelets. J Biol Chem 1986; 261: 12604–9.
- 18 Wilson E, Olcott MC, Bell RM et al. Inhibition of the oxidative burst in human neutrophils by sphingoid long-chain bases. J Biol Chem 1986; 261: 12616–23.
- 19 Merrill AH Jr, Sereni AM, Stevens VL et al. Inhibition of phorbolester-dependent differentiation of human promyelocytic leukemic (HL-60) cells by sphinganine and other long-chain bases. J Biol Chem 1986; 261: 12610–15.
- 20 Gupta AK, Fisher GJ, Elder JT et al. Sphingosine inhibits phorbolester-induced inflammation, ornithine decarboxylase activity, and activation of protein kinase C in mouse skin. J Invest Dermatol 1988; 91: 486–91.
- 21 Williams ML, Elias PM. Heterogeneity in autosomal recessive ichthyosis. Clinical and biochemical differentiation of lamellar ichthyosis and nonbullous congenital ichthyosiform erythroderma. Arch Dermatol 1985; 121: 477–88.
- 22 Judge MR, Morse-Fisher N, Manku M, Harper JL. Quantification of n-alkanes in stratum corneum in the hereditary ichthyoses. Br J Dermatol 1992; 127: 91–6.
- 23 Bergers M, Traupe H, Dunnwald SC et al. Enzymatic distinction between two subgroups of autosomal recessive lamellar ichthyosis, J Invest Dermatol 1990; 94; 407–12.
- 24 Arnold ML, Anton-Lamprecht I, Melz-Rothfuss B, Hartschuh W. Ichthyosis congenita type III. Clinical and ultrastructural characteristics and distinction within the heterogeneous ichthyosis congenita group. Arch Dermatol Res 1988; 280; 268–78.
- 25 Kanerva L, Niemi K-M, Lauharanta J, Lassus A. New observations on the fine structure of lamellar ichthyosis and the effect of treatment with etretinate. Am J Dermatopathol 1983; 5; 555–68.
- 26 Hazell M, Marks R. Clinical, histologic, and cell kinetic discriminants between lamellar ichthyosis and nonbullous congenital ichthyosiform erythroderma. Arch Dermatol 1985; 121; 489–93.
- 27 Finlay AY. Major autosomal recessive ichthyoses, Semin Dermatol 1988; 7; 26–36.
- 28 Traupe H. New classification and tables for differential diagnosis of the ichthyoses. In; The Ichthyoses; A Guide to Clinical Diagnosis, Genetic Counselling and Therapy. Berlin ; Springer-Verlag, 1989; 9–14.
- 29 Williams ML. A new look at the ichthyoses; disorders of lipid metabolism. Pediatr Dermatol 1986; 3; 476–97.
- 30 Traupe H, Kolde G, Happle R. Autosomal dominant lamellar ichthyosis; a new skin disorder. Clin Genet 1984; 26; 457–61.
- 31 Toribio J, Redondo VF, Peteiro C et al. Autosomal dominant lamellar ichthyosis. Clin Genet 1986; 30; 122–6.
- 32 Matsui MS, Chew SL, DeLeo VA. Protein kinase C in normal human epidermal keratinocytes during proliferation and calcium-induced differentiation. J Invest Dermatol 1992; 99; 565–71.
- 33 Igarashi Y, Kitamura K, Toyokuni T et al. A specific enhancing effect of N,N-dimethylsphingosine on epidermal growth factor receptor autophosphorylation. J Biol Chem 1990; 265; 5385–9.
- 34 Zhang H, Buckley NE, Gibson K, Spiegel S. Sphingosine stimulates cellular proliferation via a protein kinase C-independent pathway. J Biol Chem 1990; 265; 76–81.
- 35 Wakita N, Nishimura K, Takigawa M. Composition of free long-chain (sphingoid) bases in stratum corneum of normal and pathologic human skin conditions. J Invest Dermatol 1992; 99; 617–22.
- 36 Lidén S, Jagell S. The Sjögren-Larsson syndrome. Int J Dermatol 1984; 23; 247–53.
