Abnormal synthesis of mannose 1-phosphate derived carbohydrates in carbohydrate-deficient glycoprotein syndrome type I fibroblasts with phosphomannomutase deficiency |
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Authors: | Korner C; Lehle L; von Figura K |
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Institution: | Georg-August-Universitat, Abt. Biochemie II, Gottingen, Germany. |
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Abstract: | In fibroblasts from five patients with carbohydrate-deficient glycoprotein
syndrome type 1, the incorporation of 2-3H] mannose into mannose
phosphates, GDP-mannose, GDP-fucose, dolichol-P-mannose, lipid- linked
oligosaccharides, and glycoprotein fraction was determined. We observed a
3- to 5-fold reduction of incorporation of radioactivity into mannose
1-phosphate, GDP-mannose, GDP-fucose, dolichol-P-mannose, and nascent
glycoproteins. The incorporation of radioactivity into mannose 6-phosphate
was normal. The formation of lipid linked oligosaccharides was only
slightly affected (</=20%), but their size was severely reduced, mostly
containing five or fewer residues. As a consequence, truncated
oligosaccharides were transferred to newly synthesized glycoproteins. The
metabolic changes can be explained by a deficiency of phosphomannomutase
activity, which was reduced to </=10% of control.
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