Lysosomal dysfunction in a mouse model of sandhoff disease leads to accumulation of ganglioside-bound amyloid-β peptide

Journal of Neuroscience

Volumn 32, Issue 15, 2012, Pages 5223-5236

  Keilani, Serene ^a   Lun, Yi ^b   Stevens, Anthony C ^c   Williams, Hadis N ^c   Sjoberg, Eric R ^c   Khanna, Richie ^b   Valenzano, Kenneth J ^b   Checler, Frederic ^d   Buxbaum, Joseph D ^a   Yanagisawa, Katsuhiko ^e   Lockhart, David J ^c   Wustman, Brandon A ^c   Gandy, Sam ^a  

^a MOUNT SINAI SCHOOL OF MEDICINE   (United States)

^b Amicus Therapeutics   (United States)

^c Amicus Therapeutics   (United States)

^d INSTITUT DE PHARMACOLOGIE MOLÉCULAIRE ET CELLULAIRE   (France)

^e NATIONAL CENTER FOR GERIATRICS AND GERONTOLOGY   (Japan)

Author keywords

[No Author keywords available]

Indexed keywords

ALPHA SYNUCLEIN; AMYLOID BETA PROTEIN; AMYLOID BETA PROTEIN [1-40]; AMYLOID BETA PROTEIN [1-42]; BETA N ACETYLHEXOSAMINIDASE; GANGLIOSIDE GM2; TAU PROTEIN; UNCLASSIFIED DRUG;

ADULT; AGED; ALZHEIMER DISEASE; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; AUTOPHAGOSOME; CARBOXY TERMINAL SEQUENCE; CHILD; CONTROLLED STUDY; ENDOSOME; ENZYME LINKED IMMUNOSORBENT ASSAY; FEMALE; FRONTAL CORTEX; GM1 GANGLIOSIDOSIS; HIPPOCAMPAL CA3 REGION; HUMAN; HUMAN TISSUE; IMMUNOHISTOCHEMISTRY; IMMUNOREACTIVITY; INFANT; KNOCKOUT MOUSE; LYSOSOME; MALE; MOUSE; NONHUMAN; PRESCHOOL CHILD; PRIORITY JOURNAL; PROTEIN LOCALIZATION; SANDHOFF DISEASE; SUBSTANTIA NIGRA; TAY SACHS DISEASE; THIN LAYER CHROMATOGRAPHY; WESTERN BLOTTING;

ADULT; ALPHA-SYNUCLEIN; AMYLOID BETA-PEPTIDES; ANIMALS; BLOTTING, WESTERN; BRAIN CHEMISTRY; CHILD, PRESCHOOL; G(M2) GANGLIOSIDE; GANGLIOSIDES; HEXOSAMINIDASE B; HUMANS; IMMUNOHISTOCHEMISTRY; INFANT; LIPID METABOLISM; LYSOSOMES; MEDULLA OBLONGATA; MICE; MICE, INBRED C57BL; MICE, KNOCKOUT; SANDHOFF DISEASE; SPINAL CORD; SUBSTANTIA NIGRA; TAU PROTEINS; YOUNG ADULT;

EID: 84859554327     PISSN: 02706474     EISSN: 15292401     Source Type: Journal    
DOI: 10.1523/JNEUROSCI.4860-11.2012     Document Type: Article

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