Báo cáo khoa học: Human acid sphingomyelinase Assignment of the disulfide bond pattern
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Human acid sphingomyelinase (haSMase, EC 3.1.4.12) catalyzes the lysosomal degradation ofsphingomyelin to ceramide and phosphorylcholine. An inherited haSMase deficiency leads toNiemann–Pick disease, a severe sphingo-lipid storage disorder. The enzyme was purified and cloned over 10 years ago. Since then, only a fewstructural properties of haSMase have been elucidated.
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