Niemann-Pick disease (Crocker's type C): A histological study of the distribution and qualitative differences fo the storage process.

Abstract:

:A histochemical study is reported of regional differences of the lipid storage in a case of Niemann-Pick disease (NPD) type C. Besides tissues known to be affected (reticulo-endothelium, hepatocytes, nervous system), storage was demonstrated in adrenal cortical spongiocytes, sweat glands, renal glomerular and tubular cells, smooth muslce, excretory tubules of some salivary glands, ependyma and in choroid plexus. In most tissues were stored sphingomyelin, cholesterol and a small amount of a glycosphingolipid. In the endothelium of cerebral and spinal vessels the main stored lipid was a glycosphingolipid. The significance of these regional differences are discussed and their study is recommended as a useful counterpart to the biochemical investigation.

journal_name

Acta Neuropathol

journal_title

Acta neuropathologica

authors

Elleder M,Jirásek A,Smíd F

doi

10.1007/BF00688393

subject

Has Abstract

pub_date

1975-12-19 00:00:00

pages

191-200

issue

3

eissn

0001-6322

issn

1432-0533

journal_volume

33

pub_type

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