Lysosomal Sphingomyelinase 1053
نویسندگان
چکیده
ISection for Medical Enzymology and Metabolism, Laboratory of Biochemistry, University of Amsterdam, P.O. Box 20151, 1000 lid Amsterdam, The Netherlands; 2Institute of Organic Chemistry and Biochemistry, University of Bonn, Gerhard-Domagkstrasse i, D-5300 Bonn, Federal Republic of Germany; and 3Developmental and Metabolic Neurology Branch, National Institute of Neurological and Communicative Diseases and Stroke, National Institutes of Health, Bethesda, MD 20205, U.S.A.
منابع مشابه
The subcellular localization of neutral sphingomyelinase in rat liver.
The subcellular distribution of neutral sphingomyelinase activity has been determined in rat liver. Neutral sphingomyelinase is present in the plasma membrane. This enzyme requires either Mg2+ or Mn2+ for full activity; these cations cannot be replaced by Co2+ or Ca2+. The plasma membrane sphingomyelinase is strongly inhibited by Hg2+. A small amount of neutral spingomyelinase activity appears ...
متن کاملPlasma Membrane Repair Is Regulated Extracellularly by Proteases Released from Lysosomes
Eukaryotic cells rapidly repair wounds on their plasma membrane. Resealing is Ca(2+)-dependent, and involves exocytosis of lysosomes followed by massive endocytosis. Extracellular activity of the lysosomal enzyme acid sphingomyelinase was previously shown to promote endocytosis and wound removal. However, whether lysosomal proteases released during cell injury participate in resealing is unknow...
متن کاملMechanism of cytosol phospholipase C and sphingomyelinase-induced lysosome destabilization.
Lysosomal disintegration may cause apoptosis, necrosis and some diseases. However, mechanisms for these events are still unclear. In this study, we measured lysosomal beta-hexosaminidase free activity, membrane potential and intralysosomal pH. The results revealed that the cytosolic extracts of rat hepatocytes could increase the lysosomal permeability to both potassium ions and protons, and osm...
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Background & Aims: Niemann-Pick disease (NPD) types A and B are rare autosomal recessive disorders that occur due to mutations in the sphingomyelin phosphodiesterase 1 (SMPD1) gene, which result in the deficiency or shortage of lysosomal acid sphingomyelinase (ASM) activity. The disorder is characterized by the accumulation of sphingomyelin in the endolysosomal sections, and patients often ...
متن کاملApolipoprotein C-III-1 activates lysosomal sphingomyelinase in vitro.
Apolipoprotein (apo)C-III-1 from human very low density lipoprotein stimulates 14-fold the activity of lysosomal sphingomyelinase from human fibroblasts. At the sphingomyelin concentrations tested, maximal stimulation was obtained with 5 microM apoC-III-1 or apoC mixture. Apolipoproteins A-I, A-II, B, and C-I conferred little or no stimulation. Sphingomyelinase was stimulated 20-fold by lysopho...
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