Miglustat improves purkinje cell survival and alters microglial phenotype in feline niemann-pick disease type C

Journal of Neuropathology and Experimental Neurology

Volumn 71, Issue 5, 2012, Pages 434-448

  Stein, Veronika M ^a,b   Crooks, Alexandra ^a   Ding, Wenge ^a   Prociuk, Maria ^a   O'Donnell, Patricia ^a   Bryan, Caroline ^a   Sikora, Tracey ^a   Dingemanse, Jasper ^c   Vanier, Marie T ^d   Walkley, Steven U ^e   Vite, Charles H ^a  

^a UNIVERSITY OF PENNSYLVANIA   (United States)

^b UNIVERSITY OF VETERINARY MEDICINE HANNOVER   (Germany)

^c ACTELION PHARMACEUTICALS LTD   (Switzerland)

^d INSERM   (France)

^e ALBERT EINSTEIN COLLEGE OF MEDICINE   (United States)

Author keywords

Animal model; Cholesterol; Feline model; Glucosylceramide synthase; Glycosphingolipid; Miglustat; Niemann Pick

Indexed keywords

B7 ANTIGEN; CD14 ANTIGEN; CD1C ANTIGEN; GANGLIOSIDE GM2; GANGLIOSIDE GM3; GLYCOSPHINGOLIPID; HERMES ANTIGEN; INTERCELLULAR ADHESION MOLECULE 1; MAJOR HISTOCOMPATIBILITY ANTIGEN CLASS 1; MAJOR HISTOCOMPATIBILITY ANTIGEN CLASS 2; MIGLUSTAT; REACTIVE OXYGEN METABOLITE;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ANTIGEN EXPRESSION; AREA UNDER THE CURVE; ARTICLE; CELL SURVIVAL; CEREBELLUM; CONTROLLED STUDY; DRUG EFFICACY; DRUG HALF LIFE; DRUG MECHANISM; EXPERIMENTAL CAT; FEMALE; LIFESPAN; LIVER; MALE; MAXIMUM PLASMA CONCENTRATION; MICROGLIA; NEUROLOGIC DISEASE; NIEMANN PICK DISEASE; NONHUMAN; PHAGOCYTOSIS; PHENOTYPE; PLASMA CONCENTRATION-TIME CURVE; PRIORITY JOURNAL; PURKINJE CELL; TIME TO MAXIMUM PLASMA CONCENTRATION;

1-DEOXYNOJIRIMYCIN; ANIMALS; ANTIGENS, CD; AREA UNDER CURVE; BRAIN; CASE-CONTROL STUDIES; CAT DISEASES; CATS; CELL SURVIVAL; CHOLESTEROL; ENZYME INHIBITORS; FOLLOW-UP STUDIES; GANGLIOSIDOSES, GM2; MICROGLIA; NIEMANN-PICK DISEASE, TYPE C; PHAGOCYTOSIS; PHENOTYPE; POSTMORTEM CHANGES; PURKINJE CELLS; REACTIVE OXYGEN SPECIES; SPHINGOMYELINS; SPHINGOSINE; TIME FACTORS;

EID: 84860224053     PISSN: 00223069     EISSN: 15546578     Source Type: Journal    
DOI: 10.1097/NEN.0b013e31825414a6     Document Type: Article

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