Cerebroside 3-sulfate, accumulation

Cerebrosides other than sulfatides are decreased ceramide dihexoside sulfate accumulates. 

In addition to the ordinary cerebrosides already described, the brain contains cerebroside sulfates, or sul-fatides—lipid esters composed of a ceramide galactose and sulfate. The exact structure of the sulfatides is not known, but it has been proposed that the carbon 3 of galactose is involved in the formation of an ester bond with sulfuric acid. Sulfatides have been found in a number of tissues (liver, kidney, lung, heart, muscle, spleen), but brain seems to contain the highest concentration of sulfatides. Sulfatides are likely to accumulate in two forms of hereditary lipoidosis met-achromatic leukodystrophy and Fabry s disease. Both these diseases are rare and will be considered only briefly. 

Other authors have confirmed the radioactive sulfate incorporation data (Green and Robinson, 1960 Bakke and CoRNATZER, 1961 Davison and Gregson, 1962 Kataoka, 1962 and Burton, unpublished data). While Burton (unpublished data) has obtained results consistent with those of Rad in s for the persistence of radioactive sulfate in the cerebroside sulfate fraction. Burton s experiments indicate the same rate of loss of radioactivity due to in the carbohydrate moieties of cerebroside and cerebroside sulfate whereas Radin et al. found a retension of as well as Hauser (1964) and others (Bakke and Cornatzer, 1961, Davison and Gregson, 1962) have observed that the cerebroside cerebroside sulfate ratio in brain tissue appears to remain constant even though these lipids accumulate with age. Thus Hauser (1964) found ratios of 3 in 20 day-old rats and 4 in adult animals. Further experiments need to be carried out to determine... 

Cerebrosides and sphingomyelin are believed to accumulate in the globoid bodies. In fact, the injection of cerebrosides into rats has led to the appearance in the white matter of cells that resemble globoid cells. The biochemical defect in Krabbe s disease is still unknown, but two clues are available. There are no defects in sphingomyelin breakdown, there is a shift in the ratio of cerebrosides to sulfatides (from 3 to 1 in the normal individuals to 12 to 1 in those with leukodystrophy. These observations have led to the suggestion [127-130] that the lipidosis results from a deficiency of a sulfate-transferring enzyme (see Fig. 3-43). 

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