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January 1965, Vol 91, No. 1 >
Article | January 1965

LIPIDS IN FABRY'S DISEASE

Phillip Frost, MD
Arch Dermatol. 1965;91(1):95. doi:10.1001/archderm.1965.01600070101029.
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ABSTRACT

To the Editor:  In the Transactions of the Minnesota Dermatological Society recorded in the September, 1964, issue of the Archives of Dermatology, Dr. John Opitz, in his discussion of two cases of Fabry's disease, states that "Paper lipid chromatography and other detailed chemical studies have revealed the presence of a complex mixture of closely related choline and inositol containing aminophospholipids." I believe that more recent work has shown that the stored chemical in tissue of patients with Fabry's disease is more likely glycolipid. A careful chemical study by Sweeley and Klionsky [J Biol Chem 238:3148 (Sept) 1963] of the lipids of a kidney taken at autopsy from a patient whose symptoms and history were classical for Fabry's disease, showed that the main glycolipid stored is a ceramide trihexoside (ceramide-glucose-galactose-galactose). Lesser amounts of ceramide dihexoside were also found (ceramide-galactose-galactose).

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