A symptomatic Fabry disease mouse model generated by inducing globotriaosylceramide synthesis

Biochemical Journal

Volumn 456, Issue 3, 2013, Pages 373-383

  Taguchi, Atsumi ^a   Maruyama, Hiroki ^b   Nameta, Masaaki ^c   Yamamoto, Tadashi ^c   Matsuda, Junichiro ^d   Kulkarni, Ashok B ^e   Yoshioka, Hidekatsu ^a   Ishii, Satoshi ^a,f  

^a OITA UNIVERSITY   (Japan)

^b NIIGATA UNIVERSITY GRADUATE SCHOOL OF MEDICAL AND DENTAL SCIENCES   (Japan)

^c NIIGATA UNIVERSITY   (Japan)

^d NATIONAL INSTITUTE OF BIOMEDICAL INNOVATION   (Japan)

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

^f Biochemical Laboratory   (Japan)

Author keywords

Enzyme replacement therapy; Fabry disease; Globotriaosylceramide synthesis; Symptomatic mouse model

Indexed keywords

AGALSIDASE BETA; ALBUMIN; ALPHA GALACTOSIDASE; CREATININE; GLOBOTRIAOSYLCERAMIDE; CERAMIDE TRIHEXOSIDE; GALACTOSYLTRANSFERASE; UDP-GALACTOSE-LACTOSYLCERAMIDE ALPHA 1-4-GALACTOSYLTRANSFERASE;

ALBUMINURIA; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CATARACT; CONTROLLED STUDY; CREATININE BLOOD LEVEL; CREATININE URINE LEVEL; CROSS BREEDING; ENZYME REPLACEMENT; FABRY DISEASE; FEMALE; HEART WEIGHT; HIGH PERFORMANCE THIN LAYER CHROMATOGRAPHY; KIDNEY FUNCTION; KIDNEY MASS; KIDNEY PROXIMAL TUBULE; LIPOGENESIS; LIVER WEIGHT; MALE; MOUSE; NONHUMAN; PATHOGENESIS; PHENOTYPE; POLYURIA; PRIORITY JOURNAL; SPLEEN WEIGHT; TRANSGENIC MOUSE; UREA NITROGEN BLOOD LEVEL; URINALYSIS; URINE OSMOLALITY; URINE VOLUME; ANIMAL; BIOSYNTHESIS; DISEASE MODEL; GENETICS; HUMAN; METABOLISM; PATHOLOGY;

ALBUMINURIA; ALPHA-GALACTOSIDASE; ANIMALS; DISEASE MODELS, ANIMAL; FABRY DISEASE; GALACTOSYLTRANSFERASES; HUMANS; MICE; MICE, TRANSGENIC; TRIHEXOSYLCERAMIDES;

EID: 84888392890     PISSN: 02646021     EISSN: 14708728     Source Type: Journal    
DOI: 10.1042/BJ20130825     Document Type: Article

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