PMID: 1211110Dec 19, 1975Paper

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

Acta Neuropathologica
M EllederF Smíd

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.

References

Jan 1, 1975·Virchows Archiv. A, Pathological Anatomy and Histology·M EllederR Kohn
Dec 31, 1973·Histochemie. Histochemistry. Histochimie·M Elleder, Z Lojda
Oct 12, 1973·Clinica Chimica Acta; International Journal of Clinical Chemistry·E G BrunngraberV Zambotti
May 20, 1974·Biochemical and Biophysical Research Communications·J W CallahanJ Gerrie
Feb 1, 1972·The Journal of Pathology·M I SkikneI Webster
Jan 1, 1971·Histochemie. Histochemistry. Histochimie·M Elleder, Z Lojda
Feb 1, 1966·Proceedings of the National Academy of Sciences of the United States of America·R O BradyD S Fredrickson
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Jul 1, 1956·The Journal of Pathology and Bacteriology·O C LLOYDH URICH
Apr 1, 1961·Journal of Neurochemistry·A C CROCKER
Jul 1, 1962·Journal of Clinical Pathology·M WOLMAN

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Citations

Apr 17, 1978·Virchows Archiv. A, Pathological Anatomy and Histology·M EllederD Schlegerová
Oct 1, 1987·American Journal of Medical Genetics·L A BarnessE F Gilbert
Jul 1, 1981·European Journal of Pediatrics·E F GilbertJ M Opitz
Jan 1, 1983·Journal of Inherited Metabolic Disease·S F DonagheyJ E Crossley
Jun 25, 2009·ChemMedChem·Juha-Matti AlakoskelaPaavo K J Kinnunen
Sep 1, 1989·Pathology, Research and Practice·M Elleder

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