Literature DB >> 4160108

Metachromatic leucodystrophy: isolation and chemical analysis of metachromatic granules.

K Suzuki, G Chen.   

Abstract

The abnormal, cytoplasmic metachromatic granules found in the brain of metachromatic leucodystrophy were isolated in high purity. They show metachromasia by von Hirsch-Peiffer's cresyl violet method. Electron microscopy revealed very slight contaminations by other components, such as mitochondria, myelin, and ribosomes. Chemical analysis of two separately obtained collections has shown that the molar ratio of cholesterol to galactolipids to phosphatides is 1 : 1 : 1. Most of the galactolipids are sulfatides.

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Year:  1966        PMID: 4160108     DOI: 10.1126/science.151.3715.1231

Source DB:  PubMed          Journal:  Science        ISSN: 0036-8075            Impact factor:   47.728


  5 in total

1.  Direct tandem mass spectrometric profiling of sulfatides in dry urinary samples for screening of metachromatic leukodystrophy.

Authors:  Ladislav Kuchař; Befekadu Asfaw; Helena Poupětová; Jitka Honzíková; František Tureček; Jana Ledvinová
Journal:  Clin Chim Acta       Date:  2013-07-06       Impact factor: 3.786

Review 2.  Twenty five years of the "psychosine hypothesis": a personal perspective of its history and present status.

Authors:  K Suzuki
Journal:  Neurochem Res       Date:  1998-03       Impact factor: 3.996

3.  [Clinical and histological diagnosis of a case of familial adult metachromatic leucodystrophy (author's transl)].

Authors:  E Czmok; F Regli; A Bischoff; K Harzer; H U Benz
Journal:  J Neurol       Date:  1974       Impact factor: 4.849

4.  [Electron microscopic and neurochemical study of Alexander's disease (author's transl)].

Authors:  R Escourolle; C de Baecque; F Gray; N Baumann; J J Hauw
Journal:  Acta Neuropathol       Date:  1979-02-15       Impact factor: 17.088

5.  [Amaurotic idiocy connected with metachromatic leukodystrophy: transitional form or combination? Electron microscopic and histochemical finding].

Authors:  A Bischoff; J Ulrich
Journal:  Acta Neuropathol       Date:  1967-05-05       Impact factor: 17.088

  5 in total

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