[Metabolic disorders in patients with primary carbohydrate deficient glycoprotein syndrome]

K Zwierz 1 , A T Midro

1. Zakład Biochemii Farmaceutycznej, Instytutu Chemii, Chorób Kobiecych AkademiiMedycznej w Białymstoku.

Published:

GICID: 01.3001.0000.3130

Available language versions: en pl

Issue: Postepy Hig Med Dosw 1997; 51 (2)

Abstract

The carbohydrate-deficient glycoprotein syndromes are multisystemic inherited diseases with severe nervous system involvement. There is indirect evidence for deficiency of phosphomannomutase in type I and direct evidence for a deficiency of N-acetylglucosaminyltransferase II in type II. The disease is characterized by carbohydrate deficiencies of a number glycoproteins, including serum sialotransferrins.

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