Inhibition of GM3 synthase attenuates neuropathology of Niemann-Pick disease type C by affecting sphingolipid metabolism

Molecules and Cells

Volumn 37, Issue 2, 2014, Pages 161-171

  Lee, Hyun ^a   Lee, Jong Kil ^a,b   Bae, Yong Chul ^a   Yang, Song Hyun ^c   Okino, Nozomu ^d   Schuchman, Edward H ^e   Yamashita, Tadashi ^a,f   Bae, Jae Sung ^a,b   Jin, Hee Kyung ^a  

^a Kyungpook National University   (South Korea)

^b Kyungpook National University School of Medicine   (South Korea)

^c Institute of Metabolism   (South Korea)

^d KYUSHU UNIVERSITY   (Japan)

^e MOUNT SINAI SCHOOL OF MEDICINE   (United States)

^f HOKKAIDO UNIVERSITY   (Japan)

Author keywords

GM3; Neuropathology; Niemann pick type C disease; Sphingolipids

Indexed keywords

CHOLESTEROL; GANGLIOSIDE GM2; GANGLIOSIDE GM3; GLIAL FIBRILLARY ACIDIC PROTEIN; SPHINGOLIPID; SPHINGOMYELIN; HAEMATOSIDE SYNTHETASE; SIALYLTRANSFERASE;

ANIMAL EXPERIMENT; ANIMAL TISSUE; ARTICLE; BRAIN; BRAIN CELL CULTURE; BRAIN CORTEX; CEREBELLUM; CORPUS CALLOSUM; DEMYELINATING NEUROPATHY; DEMYELINATION; ELECTRON MICROSCOPY; ENZYME INHIBITION; HISTOPATHOLOGY; HOMOZYGOTE; IMMUNOCYTOCHEMISTRY; IMMUNOHISTOCHEMISTRY; LIPID METABOLISM; MOTOR COORDINATION; MOTOR DYSFUNCTION; MOUSE; NERVE FIBER GROWTH; NEUROPATHOLOGY; NIEMANN PICK DISEASE; NONHUMAN; OLIGODENDROGLIA; PHENOTYPE; PURKINJE CELL; THIN LAYER CHROMATOGRAPHY; WESTERN BLOTTING; ANIMAL; BAGG ALBINO MOUSE; CELL CULTURE; DISEASE MODEL; ENZYMOLOGY; GENE DELETION; GENETICS; HUMAN; METABOLISM; PATHOLOGY; TRANSGENIC MOUSE;

ANIMALS; CELLS, CULTURED; CEREBELLUM; CEREBRUM; DISEASE MODELS, ANIMAL; G(M3) GANGLIOSIDE; GENE DELETION; HUMANS; MICE, INBRED BALB C; MICE, TRANSGENIC; NIEMANN-PICK DISEASE, TYPE C; SIALYLTRANSFERASES;

EID: 84901463752     PISSN: 10168478     EISSN: 02191032     Source Type: Journal    
DOI: 10.14348/molcells.2014.2347     Document Type: Article

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