Niemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrin

Journal of Lipid Research

Volumn 55, Issue 8, 2014, Pages 1609-1621

  Vance, Jean E ^a   Karten, Barbara ^b  

^a UNIVERSITY OF ALBERTA   (Canada)

^b DALHOUSIE UNIVERSITY   (Canada)

Author keywords

Astrocytes; Cholesterol homeostasis; Endoplasmic reticulum; Gangliosides; Lysosomal storage disease; Microglia; Neurodegenerative disease; Neurons; Purkinje cells

Indexed keywords

3ALPHA HYDROXY 5ALPHA PREGNAN 20 ONE; CELL MEMBRANE PROTEIN; CHOLESTEROL; CYCLODEXTRIN; HISTONE DEACETYLASE INHIBITOR; NIEMANN PICK TYPE C 1 PROTEIN; NIEMANN PICK TYPE C 2 PROTEIN; UNCLASSIFIED DRUG; CARRIER PROTEIN; GLYCOPROTEIN; MEMBRANE PROTEIN; NPC1 PROTEIN, HUMAN; NPC2 PROTEIN, HUMAN;

CELLULAR DISTRIBUTION; CHOLESTEROL TRANSPORT; CONTINUOUS INFUSION; DISEASE MODEL; DOSE RESPONSE; DRUG EFFICACY; DRUG POTENTIATION; ENDOSOME; ENZYME INHIBITION; GENE OVEREXPRESSION; HUMAN; LIPID STORAGE; LOSS OF FUNCTION MUTATION; LOW DRUG DOSE; LYSOSOME; NEOPLASM; NERVE DEGENERATION; NEUROPATHOLOGY; NIEMANN PICK DISEASE; NONHUMAN; NPC1 GENE; NPC2 GENE; PRIORITY JOURNAL; PROTEIN DEFECT; PROTEIN DEFICIENCY; PROTEIN EXPRESSION; REVIEW; SINGLE DRUG DOSE; ANIMAL; GENETICS; METABOLISM; PATHOLOGY;

ANIMALS; CARRIER PROTEINS; CHOLESTEROL; CYCLODEXTRINS; GLYCOPROTEINS; HUMANS; LYSOSOMES; MEMBRANE GLYCOPROTEINS; NIEMANN-PICK DISEASE, TYPE C;

EID: 84905033032     PISSN: 00222275     EISSN: 15397262     Source Type: Journal    
DOI: 10.1194/jlr.R047837     Document Type: Review

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