Improvement in lipid and protein trafficking in Niemann-Pick C1 cells by correction of a secondary enzyme defect

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Volumn 11, Issue 5, 2010, Pages 601-615

  Devlin, Cecilia ^a   Pipalia, Nina H ^b   Liao, Xianghai ^a   Schuchman, Edward H ^c   Maxfield, Frederick R ^b   Tabas, Ira ^a  

^a Och Spine at New York Presbyterian Hospitals   (United States)

^b WEILL CORNELL MEDICAL COLLEGE   (United States)

^c MOUNT SINAI SCHOOL OF MEDICINE   (United States)

Author keywords

Acid sphingomyelinase; Cholesterol; Lipid trafficking; Lysosomal storage disease; Lysosomes; Niemann Pick C

Indexed keywords

CELL PROTEIN; CHOLESTEROL; LIPID; MONOACYLGLYCEROL; NIEMANN PICK C1 PROTEIN; PHOSPHORUS DERIVATIVE; SPHINGOMYELIN PHOSPHODIESTERASE; SPHINGOMYELIN PHOSPHODIESTERASE 1; TRANSFERRIN RECEPTOR; UNCLASSIFIED DRUG;

ANIMAL CELL; ARTICLE; CONTROLLED STUDY; ENZYME ACTIVITY; ENZYME DEFECT; FIBROBLAST; GENETIC DISORDER; GENETIC TRANSFECTION; HUMAN; HUMAN CELL; LIPID TRANSPORT; LYSOSOME; LYSOSOME STORAGE DISEASE; NIEMANN PICK DISEASE; NONHUMAN; PRIORITY JOURNAL; PROTEIN TRANSPORT;

ANIMALS; ANTIGENS, CD; CHO CELLS; CHOLESTEROL; CRICETINAE; CRICETULUS; FIBROBLASTS; HUMANS; LIPIDS; LYSOSOMES; NIEMANN-PICK DISEASE, TYPE C; NIEMANN-PICK DISEASES; PROTEIN TRANSPORT; PROTEINS; RECEPTORS, TRANSFERRIN; SPHINGOMYELIN PHOSPHODIESTERASE; TRANSFECTION;

EID: 77953142003     PISSN: 13989219     EISSN: 16000854     Source Type: Journal    
DOI: 10.1111/j.1600-0854.2010.01046.x     Document Type: Article

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