Increased globotriaosylceramide levels in a transgenic mouse expressing human α1,4-galactosyltransferase and a mouse model for treating Fabry disease

Journal of Biochemistry

Volumn 149, Issue 2, 2011, Pages 161-170

  Shiozuka, Chikara ^a   Taguchi, Atsumi ^a,b   Matsuda, Junichiro ^c   Noguchi, Yoko ^c   Kunieda, Takanori ^c   Uchio Yamada, Kozue ^c   Yoshioka, Hidekatsu ^b   Hamanaka, Ryoji ^b   Yano, Shinji ^b   Yokoyama, Shigeo ^b   Mannen, Kazuaki ^d   Kulkarni, Ashok B ^e   Furukawa, Koichi ^f   Ishii, Satoshi ^a,b,g  

^a OBIHIRO UNIVERSITY OF AGRICULTURE AND VETERINARY MEDICINE   (Japan)

^b OITA UNIVERSITY   (Japan)

^c NATIONAL INSTITUTE OF BIOMEDICAL INNOVATION   (Japan)

^d OITA UNIVERSITY   (Japan)

^e NATIONAL INSTITUTE OF DENTAL AND CRANIOFACIAL RESEARCH   (United States)

^f NAGOYA UNIVERSITY SCHOOL OF MEDICINE   (Japan)

^g Biochemical Laboratory   (Japan)

Author keywords

active site specific chaperone therapy; Fabry disease; globotriaosylceramide; mouse model

Indexed keywords

ALPHA 1,4 GALACTOSYLTRANSFERASE; GALACTOSYLTRANSFERASE; GLOBOTRIAOSYLCERAMIDE; MIGALASTAT; UNCLASSIFIED DRUG;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BRAIN; CONTROLLED STUDY; CROSS BREEDING; EMBRYO; ENZYME ACTIVITY; FABRY DISEASE; FEMALE; HEART; KIDNEY; LIVER; LUNG; MALE; MOUSE; MUSCLE; NONHUMAN; PROTEIN EXPRESSION; SMALL INTESTINE; SPLEEN; TRANSGENIC MOUSE;

1-DEOXYNOJIRIMYCIN; ALPHA-GALACTOSIDASE; ANIMALS; CROSSES, GENETIC; DISEASE MODELS, ANIMAL; ENZYME ACTIVATION; FABRY DISEASE; FEMALE; GALACTOSYLTRANSFERASES; HUMANS; KIDNEY; LIVER; MICE; MICE, KNOCKOUT; MICE, TRANSGENIC; MOLECULAR CHAPERONES; SPLEEN; TRIHEXOSYLCERAMIDES; UP-REGULATION;

MUS; MUS MUSCULUS;

EID: 79251501956     PISSN: 0021924X     EISSN: 17562651     Source Type: Journal    
DOI: 10.1093/jb/mvq125     Document Type: Article

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