Journal of Lipid Research, Vol. 10, 515-520, September 1969
Copyright © 1969 by Lipid Research, Inc.

Tissue distribution of glycosphingolipids in a case of Fabry's disease

James M. Schibanoff , Shigehiko Kamoshita , and John S. O'Brien

Department of Pathology, University of Southern California, School of Medicine, Los Angeles, California 90033, and the Department of Pathology, Children's Hospital, Los Angeles, California 90033

A survey was made of the glycolipid composition of various tissues, including liver, spleen, kidney (cortex and medulla), lymph node, pancreas, prostate gland, heart muscle, thenar muscle, gastrointestinal smooth muscle, frontal cerebral cortex, anterior thalamus, brain stem, a peripheral autonomic ganglion, and renal arterial intima and media, from a patient who died with Fabry's disease. The tissues had been fixed in formalin for 3 yr. Analytical data on trihexosyl ceramide from heart muscle and pancreas indicate a structure identical to trihexosyl ceramide from kidney: galactosylgalactosylglucosyl ceramide. Fatty acid compositions of trihexosyl ceramide and dihexosyl ceramide revealed a wide range of fatty acids, with 16:0, 18:0, 20:0, 22:0, 24:0, and 24:1 predominating. These glycolipids comprised 10-41% of the total lipid in the formalin-fixed organs studied. Trihexosyl ceramide predominated in all tissues and was the only glycolipid found in muscle tissues, lymph node, and arterial tissues. Dihexosyl ceramide was found in kidney, pancreas, liver, spleen, and cerebral tissues. The accumulation of trihexosyl ceramide in cardiac muscle and arterial tissues may account in part for the cardiovascular complications so prominent in Fabry's disease.

Supplementary key words trihexosyl ceramide • dihexosyl ceramide • organs • muscular tissue • nervous tissue

Submitted on February 11, 1968
Accepted on May 29, 1969

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