Accumulation of glycosphingolipids in Niemann-Pick C disease disrupts endosomal transport

Journal of Biological Chemistry

Volumn 279, Issue 25, 2004, Pages 26167-26175

  Te Vruchte, Danielle ^a   Lloyd Evans, Emyr ^a   Veldman, Robert Jan ^b   Neville, David C A ^a   Dwek, Raymond A ^a   Platt, Frances M ^a   Van Blitterswijk, Wim J ^b   Sillences, Dan J ^a  

^a UNIVERSITY OF OXFORD   (United Kingdom)

^b NETHERLANDS CANCER INSTITUTE   (Netherlands)

Author keywords

[No Author keywords available]

Indexed keywords

CELLS; CHOLESTEROL; DISEASE CONTROL; LIPIDS; PROTEINS; SUGARS;

GOLGI APPARATUS; LYSOSOMES;

BIOCHEMISTRY;

3BETA (2 DIETHYLAMINOETHOXY)ANDROST 5 EN 17 ONE; ANNEXIN; ANNEXIN 6; CHOLESTEROL; FLUORESCENT DYE; GLYCOSPHINGOLIPID; IMINO ACID; IMINOSUGAR; LACTOSYLCERAMIDE; LIPOCORTIN 2; MEMBRANE PROTEIN; N BUTYLDEOXYGLACTONOJIRIMYCIN; PROGESTERONE; REAGENT; SPHINGOSINE; SUGAR ACID; TRITON X 100; UNCLASSIFIED DRUG;

ANIMAL CELL; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CELL MEMBRANE; CHO CELL; CONTROLLED STUDY; ENDOCYTOSIS; ENDOSOME; ENZYME INHIBITION; ENZYME SUBSTRATE; GOLGI COMPLEX; HIGH PERFORMANCE LIQUID CHROMATOGRAPHY; INTRACELLULAR TRANSPORT; LIPIDOSIS; LIVER; LYSOSOME; MASS SPECTROMETRY; MOUSE; NIEMANN PICK DISEASE; NONHUMAN; PRIORITY JOURNAL; PROTEIN DOMAIN; PROTEIN FUNCTION; SPLEEN; SUBSTRATE REDUCTION THERAPY; SUCROSE DENSITY GRADIENT CENTRIFUGATION; THERAPY;

1-DEOXYNOJIRIMYCIN; ANDROSTENES; ANIMALS; ANTIGENS, CD; BIOLOGICAL TRANSPORT; BORON COMPOUNDS; BRAIN; CELL LINE; CENTRIFUGATION, DENSITY GRADIENT; CHO CELLS; CHOLESTEROL; CHROMATOGRAPHY, HIGH PRESSURE LIQUID; CRICETINAE; DETERGENTS; ENDOSOMES; ENZYME INHIBITORS; GLUCOSYLTRANSFERASES; GLYCOSPHINGOLIPIDS; GOLGI APPARATUS; LACTOSYLCERAMIDES; MASS SPECTROMETRY; MEMBRANE MICRODOMAINS; MICE; MICE, TRANSGENIC; MODELS, BIOLOGICAL; NIEMANN-PICK DISEASES; OCTOXYNOL; PHENOTYPE; PROGESTERONE; SUCROSE; ULTRACENTRIFUGATION;

ANIMALIA; CRICETINAE; CRICETULUS GRISEUS;

EID: 2942644795     PISSN: 00219258     EISSN: None     Source Type: Journal    
DOI: 10.1074/jbc.M311591200     Document Type: Article

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