全身性神经节苷脂沉积症：从黏多糖和糖蛋白中半乳糖的裂解受损。
Generalized gangliosidosis: impaired cleavage of galactose from a mucopolysaccharide and a glycoprotien.
作者信息
MacBrinn M C, Okada S, Ho M W, Hu C C, O'Brien J S
出版信息
Science. 1969 Feb 28;163(3870):946-7. doi: 10.1126/science.163.3870.946.
PMID:4236799
Abstract
We have demonstrated a tenfold (or greater) reduction of the cleavage of galactose from a mucopolysaccharide and a glycoprotein in generalized gangliosidosis, a lysosomal beta-galactosidase deficiency disease. The bony abnormalities in this disease may be due to defective catabolism of mucopolysaccharides in connective tissue.
摘要
我们已经证明，在全身性神经节苷脂沉积症（一种溶酶体β-半乳糖苷酶缺乏症）中，黏多糖和糖蛋白中半乳糖的裂解减少了十倍（或更多）。这种疾病中的骨骼异常可能是由于结缔组织中黏多糖的分解代谢缺陷所致。
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