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Urinary oligosaccharide excretion in disorders of glycolipid,glycoprotein and glycogen metabolism
Authors:A. C. Sewell
Affiliation:(1) Department of Paediatrics, University Children's Hospital, Langebeckstrasse 1, D-6500 Mainz, Federal Republic of Germany
Abstract:In recent years great interest has centered around metabolic disorders in which excessive oligosacchariduria is a prominent feature. This review describes the methods of both structural and diagnostic investigations of oligosaccharides in a number of these diseases. Special emphasis has been laid upon simple screening methods which would avail themselves to the clinical chemistry laboratory.Abbreviations Asn Asparagine - Fuc Fucose - Gal Galactose - GalNAc N-acetylgalactosamine - Glc Glucose - Man Mannose - NANA N-acetylneuraminic acid (Sialic acid)Curriculum viate. Adrian Sewell was born 1950 in Salisbury, UK. He graduated 1972 from the Biochemistry Faculty of Bath University and proceeded to the Department of Child Health at Bristol University to research for a PhD (under Dr. C. A. Pennock). Thesis Bath University 1976: Cartilage chemistry in bone dysplasias. Since 1977 he has been a research associate in the department of Pediatrics at the University Children's Hospital in Mainz (Director: Prof. Jürgen Spranger). Research interests are screening for inborn errors of metabolism, with particular reference to carbohydrate disorders, and the biochemistry of skeletal dysplasias.Supported by a grant from the Stifung Volkswagenwerk
Keywords:Oligosaccharides  Urine  Screening  Review
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