Biosynthesis of human α-N-acetylgalactosaminidase: Defective phosphorylation and maturation in infantile α-NAGA deficiency
1991
Abstract The biosynthesis of human α-N-acetylgalactosaminidase (α-NAGA) was studied in normal fibroblasts and in cells from patients with infantile α-NAGA deficiency. Normal α-NAGA is synthesized as a 52 kDa precursor which matures to a 49 kDa species through phosphorylation and carbohydrate trimming. Fibroblasts from the patients synthesize normal amounts of a 52 kDa precursor, however phosphorylation does not occur and this precursor is subsequently degraded intracellularly.
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