Iminosugar-Based inhibitors of glucosylceramide Synthase increase brain glycosphingolipids and survival in a mouse model of Sandhoff disease

PLoS ONE

Volumn 6, Issue 6, 2011, Pages

  Ashe, Karen M ^a   Bangari, Dinesh ^a   Li, Lingyun ^a   Cabrera Salazar, Mario A ^a   Bercury, Scott D ^a   Nietupski, Jennifer B ^a   Cooper, Christopher G F ^a   Aerts, Johannes M F G ^b   Lee, Edward R ^a   Copeland, Diane P ^a   Cheng, Seng H ^a   Scheule, Ronald K ^a   Marshall, John ^a  

^a GENZYME CORPORATION   (United States)

^b UNIVERSITY OF AMSTERDAM   (Netherlands)

Author keywords

[No Author keywords available]

Indexed keywords

CERAMIDE GLUCOSYLTRANSFERASE; CERAMIDE GLUCOSYLTRANSFERASE INHIBITOR; GENZ 529468; GLYCOSPHINGOLIPID; GLYCOSYLTRANSFERASE; MIGLUSTAT; UNCLASSIFIED DRUG; ENZYME INHIBITOR; GLUCOSYLTRANSFERASE; IMINOSUGAR;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BRAIN LEVEL; CONTROLLED STUDY; COORDINATION DISORDER; DRUG EFFICACY; DRUG MECHANISM; ENZYME SUBSTRATE; LIFESPAN; LIPID STORAGE; LOCOMOTION; LONGEVITY; MOTOR DYSFUNCTION; MOUSE; NONHUMAN; SANDHOFF DISEASE; SURVIVAL RATE; ANIMAL; BRAIN; CHEMISTRY; DRUG ANTAGONISM; DRUG EFFECT; IMMUNOHISTOCHEMISTRY; METABOLISM;

MUS;

ANIMALS; BRAIN; ENZYME INHIBITORS; GLUCOSYLTRANSFERASES; GLYCOSPHINGOLIPIDS; IMINO SUGARS; IMMUNOHISTOCHEMISTRY; MICE; SANDHOFF DISEASE;

EID: 79959658134     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0021758     Document Type: Article

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