Cerebrosides storage

Stem, J., Novikoff, A. B., and Terry, R. D., 1972, The induction of sulfatide ganglioside and cerebroside storage in organized nervous system cultures, in Sphingolipids, Sphingolipidoses and Allied Disorders (B. W. Volk and S. Aronson, eds.), pp. 651-660, Plenum Press, New York. 

CGT) and cerebroside sulfotransferase (CST) neuronal lipid storage was provoked. CGT-transgenic ASA(—/—) [CGT/ASA(—/—)] mice showed an accumulation of Cl8 0 fatty acid-containing sulfogalactosylceramide in the brain. Histochemicaly, an increase in sulfolipid storage could be detected in central and peripheral neurons of both CGT/ASA(—/—) and CST/ASA(—/—) mice compared with ASA(—/—) mice. CGT/ASA(—/—) mice developed severe neuromotor coordination deficits and weakness of hindlimbs and forelimbs. 

The basic lesion in Gaucher s disease has not yet been elucidated. The storage of cerebrosides, and their identification as glucocerebrosides have stimulated intensive studies on a possible abnormality of cerebroside metabolism. These have... 

Among the unexplained features is the storage of glycolipid by the infantile brain in same cases of GD. Here the possiblity of an additional metabolic error exists. A case (Jervis et al. 1962) where cytoside was the main splenic lipid instead of glucocerebroside (Rosenberg 1962) may represent a variant of GD where the enzymetic defect is localized to the site of cleavage of cytoside to cerebroside (see figure 7). 

In 1958, Jatzkewitz made the important discovery that the metachromatic material in leucodystrophy consists of cerebroside-sulfuric acid esters, proving that ML was indeed a lipid storage disease. The differentiation of leucodystrophies with prelipoid and metachromatic degradation products was now abolished. Recent reports by Austin (1957 a, b), Hagberg et al. (1960—1962), Sourander and SvENNERHOLM (1962), IsLER, Bischoff and Esslen (1963), Hollander and Pilz (1964), Fullerton (1964), and Jatzkewitz (1958—1965) underlined the existence of ML as a distinct cerebral sphingolipidosis. 

Fig. 1. Metachromatio leucoencephalopathy. Dentate nucleus of the cerebellum. Storage in ganglion cells of a brown metachromatio cerebroside sulfate. Stain acetic acid-cresyl violet (Reproduced by...

Recent studies in rabbit brain show a progressive physiologic increase in arylsulfatase and cerebroside-sulfatase after birth, with maximum activity upon completion of myelination. It is possible that a deficiency of the enzyme assumes significance at this time this seems relevant to the manifestation of ML after the first year of life. An interpretation of later onset, as in adult cases (Mehl and Jatzkewitz 1965 Hollander and Pilz 1964), is more difficult. Although here a different enzymic lesion is a possibility, a quantitatively less severe enzyme defect seems more likely. In a number of adult cases sulfatide storage was less than in infantile cases (Jatzkewitz et al. 1964 Svennerholm 1963), regardless of the speed of progression of the disease. 

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