scholarly journals A symptomatic Fabry disease mouse model generated by inducing globotriaosylceramide synthesis

2013 ◽  
Vol 456 (3) ◽  
pp. 373-383 ◽  
Author(s):  
Atsumi Taguchi ◽  
Hiroki Maruyama ◽  
Masaaki Nameta ◽  
Tadashi Yamamoto ◽  
Junichiro Matsuda ◽  
...  
Keyword(s):  
Mouse Model ◽  
Fabry Disease ◽  
Pathogenic Factor ◽  
Therapeutic Approaches

A symptomatic mouse model was generated by inducing globotriaosylceramide synthesis. Globotriaosylceramide accumulation is a primary pathogenic factor in the symptomatic phenotype. The new mouse model is useful for studying the pathogenesis of Fabry disease and therapeutic approaches.

scholarly journals Substrate Reduction Augments the Efficacy of Enzyme Therapy in a Mouse Model of Fabry Disease

PLoS ONE ◽  
2010 ◽  
Vol 5 (11) ◽  
pp. e15033 ◽  
Author(s):  
John Marshall ◽  
Karen M. Ashe ◽  
Dinesh Bangari ◽  
KerryAnne McEachern ◽  
Wei-Lien Chuang ◽  
...  
Keyword(s):  
Mouse Model ◽  
Fabry Disease ◽  
Enzyme Therapy ◽  
Substrate Reduction

scholarly journals Increased globotriaosylceramide levels in a transgenic mouse expressing human  1,4-galactosyltransferase and a mouse model for treating Fabry disease

2010 ◽  
Vol 149 (2) ◽  
pp. 161-170 ◽  
Author(s):  
C. Shiozuka ◽  
A. Taguchi ◽  
J. Matsuda ◽  
Y. Noguchi ◽  
T. Kunieda ◽  
...  
Keyword(s):  
Mouse Model ◽  
Fabry Disease ◽  
Transgenic Mouse

scholarly journals Decreased Nitric Oxide Bioavailability in a Mouse Model of Fabry Disease

2009 ◽  
Vol 20 (9) ◽  
pp. 1975-1985 ◽  
Author(s):  
Liming Shu ◽  
James L. Park ◽  
Jaeman Byun ◽  
Subramaniam Pennathur ◽  
Jessica Kollmeyer ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Mouse Model ◽  
Fabry Disease ◽  
Nitric Oxide Bioavailability

Gene Expression Profiling of a Mouse Model of Fabry Disease

2012 ◽  
Vol 105 (2) ◽  
pp. S27
Author(s):  
Shaalee Dworski ◽  
Salvador Mejia-Guerrero ◽  
Natalia Pacienza ◽  
Bryan Au ◽  
Jeffrey Medin
Keyword(s):  
Gene Expression ◽  
Mouse Model ◽  
Fabry Disease ◽  
Gene Expression Profiling ◽  
Expression Profiling

Effects of genetic background on disease phenotypes in a mouse model of Fabry disease

2019 ◽  
Vol 126 (2) ◽  
pp. S79
Author(s):  
Siamak Jabbarzadeh-Tabrizi ◽  
Taniqua Day ◽  
Mouna Taroua ◽  
Michel Boutin ◽  
Christiane Auray-Blais ◽  
...  
Keyword(s):  
Mouse Model ◽  
Fabry Disease ◽  
Genetic Background ◽  
Disease Phenotypes

Reduced glucosylceramide in the mouse model of Fabry disease: Correction by successful enzyme replacement therapy

Gene ◽  
2014 ◽  
Vol 536 (1) ◽  
pp. 97-104 ◽  
Author(s):  
Rui Quinta ◽  
Daniel Rodrigues ◽  
Marisa Assunção ◽  
Maria Fatima Macedo ◽  
Olga Azevedo ◽  
...  
Keyword(s):  
Mouse Model ◽  
Enzyme Replacement Therapy ◽  
Fabry Disease ◽  
Replacement Therapy ◽  
Enzyme Replacement

scholarly journals The Pharmacological Chaperone 1-Deoxygalactonojirimycin Reduces Tissue Globotriaosylceramide Levels in a Mouse Model of Fabry Disease

2010 ◽  
Vol 18 (1) ◽  
pp. 23-33 ◽  
Author(s):  
Richie Khanna ◽  
Rebecca Soska ◽  
Yi Lun ◽  
Jessie Feng ◽  
Michelle Frascella ◽  
...  
Keyword(s):  
Mouse Model ◽  
Fabry Disease ◽  
Pharmacological Chaperone

scholarly journals Local and global cerebral blood flow and glucose utilization in the α-galactosidase A knockout mouse model of Fabry disease

2002 ◽  
Vol 79 (6) ◽  
pp. 1217-1224 ◽  
Author(s):  
Yoshiaki Itoh ◽  
Takanori Esaki ◽  
Michelle Cook ◽  
Pankaj Qasba ◽  
Kazuaki Shimoji ◽  
...  
Keyword(s):  
Blood Flow ◽  
Cerebral Blood Flow ◽  
Mouse Model ◽  
Fabry Disease ◽  
Knockout Mouse ◽  
Glucose Utilization ◽  
Knockout Mouse Model ◽  
Global Cerebral Blood Flow

Priapism in a Fabry disease mouse model is associated with upregulated penile nNOS and eNOS expression

2018 ◽  
Vol 123 (2) ◽  
pp. S130
Author(s):  
Jin-Song Shen ◽  
Xing-Li Meng ◽  
Arning Erland ◽  
Taniqua Day ◽  
Siamak Jabbarzadeh-Tabrizi ◽  
...  
Keyword(s):  
Mouse Model ◽  
Fabry Disease ◽  
Enos Expression
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