Lysosomal and Mitochondrial Liaisons in Niemann-Pick Disease
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منابع مشابه
Lysosomal and Mitochondrial Liaisons in Niemann-Pick Disease
Lysosomal storage disorders (LSD) are characterized by the accumulation of diverse lipid species in lysosomes. Niemann-Pick type A/B (NPA/B) and type C diseases Niemann-Pick type C (NPC) are progressive LSD caused by loss of function of distinct lysosomal-residing proteins, acid sphingomyelinase and NPC1, respectively. While the primary cause of these diseases differs, both share common biochem...
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In the first part the properties of normal mammalian sphingomyelinases are reviewed: The lysosomal acid sphingomyelinase is a polymeric glycoprotein (subunit Mr between 28 000 and 70 000) which hydrolyses natural sphingomyelin, coloured and fluorescent semi-synthetic analogues (trinitrophenyl-aminolauryl-sphingomyelin and pyrenedecanoyl-sphingomyelin) and the synthetic analogue 2-N-hexadecanoyl...
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ژورنال
عنوان ژورنال: Frontiers in Physiology
سال: 2017
ISSN: 1664-042X
DOI: 10.3389/fphys.2017.00982