Biology of Brain Dysfunction: Volume 2 by Kunihiko Suzuki, Kinuko Suzuki (auth.), Gerald E. Gaull

By Kunihiko Suzuki, Kinuko Suzuki (auth.), Gerald E. Gaull (eds.)

The progress of neurochemistry, molecular biology, and biochemical genetics has ended in a burgeoning of recent details proper to the pathogenesis of mind disorder. This explosion of fascinating new info is crying out for collation and significant synthesis. In its totality, it defies systematic summa­ tion, and, in fact, nobody writer can cope. hence invites for contributions got to varied specialists in parts that are below energetic research, of present neurological curiosity, and pregnant. even supposing this undertaking is comparatively entire, through dint of measurement, different subject matters could have been incorporated; the choice used to be exclusively my accountability. i feel systematic summation a digital impossibility-indeed, hardly ever well worth the attempt. The try to gather all the sections fascinated with a wide treatise with a number of authors necessarily leads to untoward delays as a result of distinction within the price at which a variety of authors paintings. as a result, the next procedure has been followed: a number of small volumes and a comparatively versatile layout, with ebook so as of receipt and once sufficient chapters are assembled to make e-book functional and most economical. during this method, the time lag among the information and their emergence in print is the shortest.

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Therefore, it is understandable that a disease caused by a metabolic disturbance of lactosylceramide has both neurological and systemic manifestations. From the single case thus far reported, the disease appears to be relatively mild in neurological manifestations. Visceral accumulation of lactosylceramide is likewise much less severe than that of glucocerebroside in Gaucher's disease, despite the fact that the turnover rates of glucocerebroside and lactosylceramide should be similar since these compounds are next to each other on the degradative pathway of the same series of compounds.

Then, at least in cases where there is abnormal accumulation of glucocerebroside in the brain, the neuropathic effects may be due to such accumulation. The source of glucocerebroside in the brain is likely to be gangliosides, which are known to turn over actively. The existence of the degradative block itself rather than its consequence, accumulation of glucocerebroside, might adversely affect the regulation of ganglioside metabolism, thus causing functional disturbance of neurons. The question of the presence or absence of CNS involvement also remains for future investigation.

The recent successful trial of phenyl hydantoin as the therapeutic agent for the paroxysmal pains also indicates that the phenomenon is not merely vascular. (250) The most likely source of digalactosylglucosylceramide is globoside. Globoside is a small but normal constituent of most of the systemic tissues and is present at a relatively high concentration in erythrocytes. Its first catabolic product is digalactosylglucosylceramide. The significance of digalactosylceramide accumulation is more obscure.

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