Creation of a mouse model for non-neurological (type B) Niemann-Pick disease by stable, low level expression of lysosomal sphingomyelinase in the absence of secretory sphingomyelinase: Relationship between brain intra-lysosomal enzyme activity and central nervous system function

Human Molecular Genetics

Volumn 9, Issue 13, 2000, Pages 1967-1976

  Marathe, Sudhir ^a   Miranda, Silvia R P ^b   Devlin, Cecilia ^a   Johns, Anthony ^c   Kuriakose, George ^a   Williams, Kevin Jon ^d   Schuchman, Edward H ^b   Tabas, Ira ^a  

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

^b MOUNT SINAI SCHOOL OF MEDICINE   (United States)

^c BAYER AG   (Germany)

^d THOMAS JEFFERSON UNIVERSITY   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

BRAIN ENZYME; HYDROLASE; LYSOSOME ENZYME; SPHINGOMYELIN; SPHINGOMYELIN PHOSPHODIESTERASE;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; BRAIN TISSUE; CELL INCLUSION; CENTRAL NERVOUS SYSTEM FUNCTION; CEREBELLUM; CONTROLLED STUDY; ENZYME ACTIVITY; ENZYME ASSAY; ENZYME DEFICIENCY; FEMALE; HEALTH STATUS; KNOCKOUT MOUSE; LIFESPAN; LYSOSOME; LYSOSOME STORAGE DISEASE; MOUSE; NERVE DEGENERATION; NEUROLOGIC DISEASE; NEUROPROTECTION; NIEMANN PICK DISEASE; NONHUMAN; PRIORITY JOURNAL; PROTEIN EXPRESSION; PROTEIN TARGETING; PURKINJE CELL; TRANSGENIC MOUSE;

ANIMALIA; MUS MUSCULUS;

EID: 0034641594     PISSN: 09646906     EISSN: None     Source Type: Journal    
DOI: 10.1093/hmg/9.13.1967     Document Type: Article

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