Substrate reduction reduces gangliosides in postnatal cerebrum-brainstem and cerebellum in GM1 gangliosidosis mice

Journal of Lipid Research

Volumn 46, Issue 4, 2005, Pages 744-751

  Kasperzyk, J L ^a   D'Azzo, A ^a   Platt, F M ^a   Alroy, J ^a   Seyfried, T N ^a  

^a BOSTON COLLEGE   (United States)

Author keywords

Asialo derivative; Cerebrosides; Granule cells; II3 (NeuAc)2 LacCer; II3NeuAc GgOse4Cer; Myelin; N butyldeoxygalactonojirimycin; Neurodegeneration

Indexed keywords

CEREBROSIDE; GANGLIOSIDE; IMINO ACID; N BUTYLDEOXYGALACTO NOJIRIMYCIN; NOJIRIMYCIN; UNCLASSIFIED DRUG; 1 DEOXYNOJIRIMYCIN; DRUG DERIVATIVE; N BUTYLDEOXYGALACTONOJIRIMYCIN; N-BUTYLDEOXYGALACTONOJIRIMYCIN; SPHINGOMYELIN;

ANIMAL TISSUE; ARTICLE; BIOSYNTHESIS; BODY WEIGHT; BRAIN DEVELOPMENT; BRAIN STEM; BRAIN WEIGHT; CELL MIGRATION; CELL PROLIFERATION; CENTRAL NERVOUS SYSTEM; CEREBELLUM; CONTROLLED STUDY; GM1 GANGLIOSIDOSIS; GRANULE CELL; MOUSE; NONHUMAN; PRIORITY JOURNAL; SYNAPTOGENESIS; WATER CONTENT; ANIMAL; C57BL MOUSE; DRUG EFFECT; ENZYME SPECIFICITY; METABOLISM; MOUSE MUTANT; NEWBORN; OXIDATION REDUCTION REACTION; PATHOLOGY; THIN LAYER CHROMATOGRAPHY;

1-DEOXYNOJIRIMYCIN; ANIMALS; ANIMALS, NEWBORN; BRAIN STEM; CEREBELLUM; CHROMATOGRAPHY, THIN LAYER; GANGLIOSIDES; GANGLIOSIDOSIS, GM1; MICE; MICE, INBRED C57BL; MICE, KNOCKOUT; OXIDATION-REDUCTION; SPHINGOMYELINS; SUBSTRATE SPECIFICITY;

EID: 21744460384     PISSN: 00222275     EISSN: 15397262     Source Type: Journal    
DOI: 10.1194/jlr.M400411-JLR200     Document Type: Article

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