Glucosylceramidase Mass and Subcellular Localization Are Modulated by Cholesterol in Niemann-Pick Disease Type C

Journal of Biological Chemistry

Volumn 279, Issue 17, 2004, Pages 17674-17680

  Salvioli, Rosa ^a   Scarpa, Susanna ^b   Ciaffoni, Fiorella ^a   Tatti, Massimo ^a   Ramoni, Carlo ^a   Vanier, Marie T ^c   Vaccaro, Anna Maria ^a  

^a ISTITUTO SUPERIORE DI SANITÀ   (Italy)

^b SAPIENZA UNIVERSITY OF ROME   (Italy)

^c CENTRE HOSPITALIER LYON SUD   (France)

Author keywords

[No Author keywords available]

Indexed keywords

CHOLESTEROL; DISEASE CONTROL; ENZYMES; FLUORESCENCE; MICROSCOPIC EXAMINATION; PHOSPHOLIPIDS;

FIBROBLASTS; IMMUNOFLUORESCENCE MICROSCOPY; LIPID ACTIVATORS; VESICULAR STRUCTURES;

BIOCHEMISTRY;

CHOLESTEROL; GLUCOSYLCERAMIDASE; GLUCOSYLCERAMIDE; LYSOBISPHOSPHATIDIC ACID; LYSOPHOSPHATIDIC ACID; SPHINGOLIPID; SPHINGOLIPID ACTIVATOR PROTEIN; SPHINGOLIPID ACTIVATOR PROTEIN C; UNCLASSIFIED DRUG;

ARTICLE; CELL VACUOLE; CONTROLLED STUDY; ENDOSOME; ENZYME ACTIVITY; ENZYME LOCALIZATION; ENZYME STABILITY; FIBROBLAST; HUMAN; HUMAN CELL; IMMUNOFLUORESCENCE MICROSCOPY; LYSOSOME; MASS; NIEMANN PICK DISEASE; PRIORITY JOURNAL; PROTEIN PROTEIN INTERACTION;

BLOTTING, WESTERN; CELL LINE; CHOLESTEROL; ENDOSOMES; FIBROBLASTS; GLUCOSYLCERAMIDASE; GLYCOPROTEINS; HUMANS; LIPID METABOLISM; LYSOPHOSPHOLIPIDS; LYSOSOMES; MICROSCOPY, FLUORESCENCE; MONOGLYCERIDES; MUTATION; NIEMANN-PICK DISEASES; PHOSPHOLIPIDS; PRECIPITIN TESTS; SAPOSINS; TIME FACTORS;

EID: 2342436735     PISSN: 00219258     EISSN: None     Source Type: Journal    
DOI: 10.1074/jbc.M313517200     Document Type: Article

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