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J Biol Chem, Vol. 274, Issue 16, 11038-11045, April 16, 1999
From the The epidermal permeability barrier is maintained
by extracellular lipid membranes within the interstices of the stratum
corneum. Ceramides, the major components of these multilayered
membranes, derive in large part from hydrolysis of glucosylceramides
mediated by stratum corneum
Sphingolipid Activator Proteins Are Required for Epidermal
Permeability Barrier Formation
,,,
, and
Kekulé-Institut für Organische
Chemie und Biochemie, Universität Bonn, Gerhard-Domagk-Strasse 1, D-53121 Bonn, Germany, the § Department of Dermatology,
School of Medicine, University of California and the Dermatology
Service and Research Unit (190), Veterans Affairs Medical Center, San
Francisco, California 94121, and the Departments of Neurology
and Psychiatry, School of Medicine, University of North Carolina,
Chapel Hill, North Carolina, 27599
-glucocerebrosidase (-GlcCerase).
Prosaposin (pSAP) is a large precursor protein that is proteolytically
cleaved to form four distinct sphingolipid activator proteins, which
stimulate enzymatic hydrolysis of sphingolipids, including
glucosylceramide. Recently, pSAP has been eliminated in a mouse model
using targeted deletion and homologous recombination. In addition to
the extracutaneous findings noted previously, our present data indicate
that pSAP deficiency in the epidermis has significant consequences
including: 1) an accumulation of epidermal glucosylceramides together
with below normal levels of ceramides; 2) alterations in lipids that are bound by ester linkages to proteins of the cornified cell envelope;
3) a thickened stratum lucidum with evidence of scaling; and 4) a
striking abnormality in lamellar membrane maturation within the
interstices of the stratum corneum. Together, these results demonstrate
that the production of pSAP, and presumably mature sphingolipid
activator protein generation, is required for normal epidermal barrier
formation and function. Moreover, detection of significant amounts of
covalently bound 
-OH-GlcCer in pSAP-deficient epidermis suggests
that deglucosylation to -OH-Cer is not a requisite step prior to
covalent attachment of lipid to cornified envelope proteins.
Copyright © 1999 by The American Society for Biochemistry and Molecular Biology, Inc.
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