ISSN:
1432-0533
Keywords:
Mucopolysaccharidoses III
;
Sanfilippo's syndrome
;
Histochemistry
;
Electron microscopy
;
Lipofuscin
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Summary A histochemical and ultrastructural study was made on the brain of a 23-year-old man with Sanfilippo's syndrome. In accordance with previous reports the cortical nerve cells contained a PAS-positive lipid storage substance. This showed intense autofluorescence in UV-light and was positive with various stains for lipofuscin. The storage material appeared ultrastructurally as inclusion bodies composed of short lamellated membranes, granular material, and vacuoles. In addition, concentrically and transversely lamellated membranous cytoplasmic bodies were observed in the nerve cells. It is concluded that the PAS-positive lipid storage material in the neurons was composed partly of lipofuscin in addition to other lipids presumably glycosphingolipids.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00697002
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