Chemical chaperone treatment for galactosialidosis: Effect of NOEV on β-galactosidase activities in fibroblasts

Brain and Development

Volumn 38, Issue 2, 2016, Pages 175-180

  Hossain, Mohammad Arif ^a,d   Higaki, Katsumi ^b   Shinpo, Michiko ^a   Nanba, Eiji ^b   Suzuki, Yoshiyuki ^c   Ozono, Keiichi ^a   Sakai, Norio ^a  

^a OSAKA UNIVERSITY GRADUATE SCHOOL OF MEDICINE   (Japan)

^b TOTTORI UNIVERSITY   (Japan)

^c TOKYO METROPOLITAN INSTITUTE OF MEDICAL SCIENCE   (Japan)

^d KING SAUD BIN ABDULAZIZ UNIVERSITY FOR HEALTH SCIENCES   (Saudi Arabia)

Author keywords

Chaperone; Cherry red spot; Galactosialidosis; Hydrops fetalis; N Octyl 4 epi valienamine

Indexed keywords

2 OCTYL 4 EPI BETA VALIENAMINE; AMINE; BETA GALACTOSIDASE; CARBOXYPEPTIDASE C; SIALIDASE; UNCLASSIFIED DRUG; ACID BETA-GALACTOSIDASE; CHAPERONE; CTSA PROTEIN, HUMAN; HEXOSAMINE; N-OCTYL-BETA-VALIENAMINE;

ADOLESCENT; ADULT; ARTICLE; CASE REPORT; CELL LYSATE; CHILD; CONTROLLED STUDY; CTSA GENE; DRUG EFFECT; ENZYME STABILITY; FETUS; FIBROBLAST; GALACTOSIALIDOSIS; GENE; GENE MUTATION; HUMAN; HUMAN CELL; IN VITRO STUDY; ONSET AGE; PRESCHOOL CHILD; SKIN FIBROBLAST; WESTERN BLOTTING; CELL CULTURE; DRUG EFFECTS; ENZYMOLOGY; GANGLIOSIDOSIS, GM1; METABOLISM; MUTATION; NEWBORN; PATHOLOGY;

ADOLESCENT; ADULT; BETA-GALACTOSIDASE; CATHEPSIN A; CELLS, CULTURED; CHILD, PRESCHOOL; FIBROBLASTS; GANGLIOSIDOSIS, GM1; HEXOSAMINES; HUMANS; INFANT, NEWBORN; MOLECULAR CHAPERONES; MUTATION;

EID: 84951568921     PISSN: 03877604     EISSN: 18727131     Source Type: Journal    
DOI: 10.1016/j.braindev.2015.07.006     Document Type: Article

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