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Tay-Sachs DiseaseDemonstration of the Stored Ganglioside in Cultured Cells From Brain Biopsy
Ulrich Batzdorf, MD;
Louis L. Sarlieve, MS;
Vivian A. Gold, BA;
John H. Menkes, MD
Arch Neurol. 1969;20(6):650-652.
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TAY-SACHS disease (infantile amaurotic idiocy) is a neuronal storage disease inherited as a simple mendelian recessive trait and characterized by progressive mental deterioration and blindness. Large quantities of a ganglioside, a neuraminic acid containing macromolecular glycolipid, are stored in almost all cells of the central nervous system (CNS). Electronmicroscopic examination of involved neurons indicates that the lipid is arranged in concentric lamellae, termed membranous cytoplasmic bodies by Terry and Weiss.1 The lipid is also encountered in reactive astrocytes, in endothelial cells, and in pericytes, which are often present in large numbers in Tay-Sachs disease, and which may act as macrophages to remove lipid debris from ruptured neurons.1 Ganglioside-laden macrophages have also been seen to surround cerebral blood vessels and may be found in the leptomeninges.
Cells from patient afflicted with various neuronal storage diseases have recently been grown in vitro, and the abnormally stored material has been
. . . [Full Text PDF of this Article]
Author Affiliations
Los Angeles
From the Department of Surgery\Neurosurgery (Dr. Batzdorf), the Division of Pediatric Neurology (Mrs. Gold, Mr. Sarlieve, and Dr. Menkes), and the Brain Research Institute (Drs. Batzdorf and Menkes), UCLA School of Medicine, Los Angeles.
Footnotes
Submitted for publication Jan 27, 1969; accepted Feb 11.
Reprint requests to the Division of Pediatric Neurology, UCLA School of Medicine, Los Angeles 90024 (Dr. Menkes).
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