Cln1 gene disruption in mice reveals a common pathogenic link between two of the most lethal childhood neurodegenerative lysosomal storage disorders

Human Molecular Genetics

Volumn 24, Issue 19, 2015, Pages 5416-5432

  Chandra, Goutam ^a   Bagh, Maria B ^a   Peng, Shiyong ^a   Saha, Arjun ^a   Sarkar, Chinmoy ^a   Moralle, Matthew ^a   Zhang, Zhongjian ^a   Mukherjee, Anil B ^a  

^a EUNICE KENNEDY SHRIVER NATIONAL INSTITUTE OF CHILD HEALTH AND HUMAN DEVELOPMENT   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ALPHA SYNUCLEIN; CATHEPSIN D; MATRIX METALLOPROTEINASE; MESSENGER RNA; SHORT HAIRPIN RNA; THIOL ESTER HYDROLASE; CTSD PROTEIN, MOUSE; HYDROXYLAMINE; N-TERT-BUTYLHYDROXYLAMINE; PALMITOYL-PROTEIN THIOESTERASE;

ANIMAL CELL; ANIMAL TISSUE; ARTICLE; ASTROCYTE; ASTROCYTE CULTURE; BRAIN CELL CULTURE; BRAIN CORTEX; CATALYSIS; CELL LYSATE; CLN1 GENE; COMPARATIVE STUDY; CONFOCAL MICROSCOPY; CONTROLLED STUDY; DIMERIZATION; ENDOSOME; ENZYME ACTIVITY; GENE; GENE DISRUPTION; GENE OVEREXPRESSION; GENETIC TRANSFECTION; HISTOPATHOLOGY; IMMUNOPRECIPITATION; IMMUNOREACTIVITY; LYSOSOME; LYSOSOME STORAGE DISEASE; MICROGLIA; MOUSE; NERVE CELL CULTURE; NEURONAL CEROID LIPOFUSCINOSIS; NEUROTOXICITY; NONHUMAN; OXIDATIVE STRESS; PATHOGENESIS; PH; POLYACRYLAMIDE GEL ELECTROPHORESIS; PRIORITY JOURNAL; PROTEIN DEGRADATION; PROTEIN DEPLETION; REAL TIME POLYMERASE CHAIN REACTION; REVERSE TRANSCRIPTION POLYMERASE CHAIN REACTION; UPREGULATION; WESTERN BLOTTING; ANIMAL; BRAIN; CHILD; DEFICIENCY; DISEASE MODEL; GENE EXPRESSION REGULATION; GENETICS; HUMAN; METABOLISM; MUTATION; NEURONAL CEROID-LIPOFUSCINOSES; PATHOLOGY;

ANIMALS; BRAIN; CATHEPSIN D; CHILD; DISEASE MODELS, ANIMAL; GENE EXPRESSION REGULATION; HUMANS; HYDROXYLAMINES; LYSOSOMES; MICE; MUTATION; NEURONAL CEROID-LIPOFUSCINOSES; THIOLESTER HYDROLASES;

EID: 84943747165     PISSN: 09646906     EISSN: 14602083     Source Type: Journal    
DOI: 10.1093/hmg/ddv266     Document Type: Article

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