The Normal Sulfatides

Until recently, therefore, the question remained unsettled whether sulfatides contained phosphorus or not. Lees et al. (1959) were able to isolate from brain white matter a sulfatide A in cristalline form, and a sulfatide B which was associated with phospholipids. Similarly Bakke and Cornatzer (1961), and Davison and Gregson (1962) found in normal human brains sulfatides with and without phosphorus upon column chromatographic separation, and the latter authors considered the phosphorus-containing component to result from mere association of sulfatides with phospholipids. In contrast. Green and Robinson (1960), upon paper-chromatography of brain lipid extracts, observed one spot only corresponding to the authentic cerebron sulfate. Today it is generally accepted that sulfatides do not contain phosphorus. 

Cerebronic acid makes up the greatest portion of brain sulfatide fatty acids according to Blix (1933). More recent analyses showed lignoceric, nervonic and oxynervonic acids also to be present (Jatzkewitz 1960b, 1963 Sekeris 1964 O Brien et al. 1964). 

With regard to the position of the sulfuric acid moiety, Nakayama (1951), on the basis of unsuccessful tritylation, suspected ester binding with the primary hydroxyl group. Thannhauser and Boncoddo (1953) and Thannhauser et al. (1955) suggested binding of the sulfuric acid moiety to the primary hydroxyl group of Cq of galactose. More recently, it could be proved that the sulfuric acid is actually bound to C3 (Yamakawa et al. 1962 Hakomori et al. 1962 Stoffyn and Stoffyn 1963 a, b Taketomi and Yamakawa 1964). 

According to information available to date, the sulfatide of nerve tissue is a sulfuric acid ester of cerebrosides and consists of one mole each of sphingosine, fatty acid, galactose and sulfuric acid. In addition, a dihexose sulfatide is found in the kidney (Martens son 1963, Martens son et al. 1966) and possibly in other tissues as well, which contains one mole glucose in addition to galactose. In this dihexose sulfatide glucose is vicinal to ceramide. 

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The accumulation of sulfatides in ML is not limited to the nervous system. Since, however, the normal sulfatide content of visceral organs is very low and measurement is difficult, data on the increase in ML vary. While Austin (1959 b) found the sulfatide content of the kidneys increased nine-fold Svennerholm (1963), from comparative studies in three normal infants (aged two months) and two patients with late-infantile ML (aged 3% and 4% years respectively) found a 25—70 fold increase in kidney sulfatides (see table 5), particularly monohexosesulfatide, above normal (1.26 and 4.60% of dry weight as compared to 0.09—0.17% normally)... 

Since the carbohydrate moiety of the monohexose-sulfatide is galactose, as in normals, it appears certain that the stored sulfatide in ML is identical with the normal sulfatide. 

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