## Abstract Fabry's disease is a lipid storage disease caused by an Xโlinked hereditary deficiency of ฮฑโgalactosidase. The enzymatic defect causes progressive deposition of ceramide trihexoside (CTH) in various tissues, leading to renal failure, premature myocardial infarction, and stroke, with a h
Total synthesis of ceramide trihexoside accumulating with fabry's disease
โ Scribed by David Shapiro; A.J. Acher
- Publisher
- Elsevier Science
- Year
- 1978
- Tongue
- English
- Weight
- 536 KB
- Volume
- 22
- Category
- Article
- ISSN
- 0009-3084
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โฆ Synopsis
We report the synthesis of natural ceramide tdhexoside, viz. gal (a-1 ~ 4) gal (#-1 --, 4) gluc (#-1 ~ 1) ceramide (XIII). It involves the Koenigs-Knorr reaction of the bromide II with the aglucon XIX, and of the chloride VII with the D-enantiomer of the ceramide ester XlI. A positional isomer of XIII was obtained as a by-product. A novel technique in the Koenings-Knorr reaction is described.
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