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Glycogen Storage Disease Type Ia in Canines : A Model for Human Metabolic and Genetic Liver Disease
Joint Authors
Lee, Young Mok
Cossette, Travis
Struck, Maggie B.
Weinstein, David A.
Mah, Cathryn S.
Verstegen, John
Fiske, Laurie
Specht, Andrew J.
Correia, Catherine E.
Byrne, Barry J.
Chou, Janice Y.
Conlon, Thomas J.
Erger, Kirsten
Campbell-Thompson, Martha
Source
Journal of Biomedicine and Biotechnology
Issue
Vol. 2011, Issue 2011 (31 Dec. 2011), pp.1-9, 9 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2011-01-03
Country of Publication
Egypt
No. of Pages
9
Main Subjects
Abstract EN
A canine model of Glycogen storage disease type Ia (GSDIa) is described.
Affected dogs are homozygous for a previously described M121I mutation resulting in a deficiency of glucose-6-phosphatase-α.
Metabolic, clinicopathologic, pathologic, and clinical manifestations of GSDIa observed in this model are described and compared to those observed in humans.
The canine model shows more complete recapitulation of the clinical manifestations seen in humans including “lactic acidosis”, larger size, and longer lifespan compared to other animal models.
Use of this model in preclinical trials of gene therapy is described and briefly compared to the murine model.
Although the canine model offers a number of advantages for evaluating potential therapies for GSDIa, there are also some significant challenges involved in its use.
Despite these challenges, the canine model of GSDIa should continue to provide valuable information about the potential for generating curative therapies for GSDIa as well as other genetic hepatic diseases.
American Psychological Association (APA)
Specht, Andrew J.& Fiske, Laurie& Erger, Kirsten& Cossette, Travis& Verstegen, John& Campbell-Thompson, Martha…[et al.]. 2011. Glycogen Storage Disease Type Ia in Canines : A Model for Human Metabolic and Genetic Liver Disease. Journal of Biomedicine and Biotechnology،Vol. 2011, no. 2011, pp.1-9.
https://search.emarefa.net/detail/BIM-487849
Modern Language Association (MLA)
Specht, Andrew J.…[et al.]. Glycogen Storage Disease Type Ia in Canines : A Model for Human Metabolic and Genetic Liver Disease. Journal of Biomedicine and Biotechnology No. 2011 (2011), pp.1-9.
https://search.emarefa.net/detail/BIM-487849
American Medical Association (AMA)
Specht, Andrew J.& Fiske, Laurie& Erger, Kirsten& Cossette, Travis& Verstegen, John& Campbell-Thompson, Martha…[et al.]. Glycogen Storage Disease Type Ia in Canines : A Model for Human Metabolic and Genetic Liver Disease. Journal of Biomedicine and Biotechnology. 2011. Vol. 2011, no. 2011, pp.1-9.
https://search.emarefa.net/detail/BIM-487849
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-487849