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Serial MRI Features of Canine GM1 Gangliosidosis : A Possible Imaging Biomarker for Diagnosis and Progression of the Disease
Joint Authors
Nakamoto, Yuya
Ozawa, Tsuyoshi
Takahashi, Kimimasa
Kuwabara, Takayuki
Hasegawa, Daisuke
Fujita, Michio
Itamoto, Kazuhito
Orima, Hiromitsu
Mizoguchi, Shunta
Yamato, Osamu
Yabuki, Akira
Source
Issue
Vol. 2012, Issue 2012 (31 Dec. 2012), pp.1-10, 10 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2012-03-12
Country of Publication
Egypt
No. of Pages
10
Main Subjects
Natural & Life Sciences (Multidisciplinary)
Medicine
Information Technology and Computer Science
Abstract EN
GM1 gangliosidosis is a fatal neurodegenerative lysosomal storage disease caused by an autosomal recessively inherited deficiency of β-galactosidase activity.
Effective therapies need to be developed to treat the disease.
In Shiba Inu dogs, one of the canine GM1 gangliosidosis models, neurological signs of the disease, including ataxia, start at approximately 5 months of age and progress until the terminal stage at 12 to 15 months of age.
In the present study, serial MR images were taken of an affected dog from a model colony of GM1 gangliosidosis and 4 sporadic clinical cases demonstrating the same mutation in order to characterize the MRI features of this canine GM1 gangliosidosis.
By 2 months of age at the latest and persisting until the terminal stage of the disease, the MR findings consistently displayed diffuse hyperintensity in the white matter of the entire cerebrum on T2-weighted images.
In addition, brain atrophy manifested at 9 months of age and progressed thereafter.
Although a definitive diagnosis depends on biochemical and genetic analyses, these MR characteristics could serve as a diagnostic marker in suspect animals with or without neurological signs.
Furthermore, serial changes in MR images could be used as a biomarker to noninvasively monitor the efficacy of newly developed therapeutic strategies.
American Psychological Association (APA)
Hasegawa, Daisuke& Yamato, Osamu& Nakamoto, Yuya& Ozawa, Tsuyoshi& Yabuki, Akira& Itamoto, Kazuhito…[et al.]. 2012. Serial MRI Features of Canine GM1 Gangliosidosis : A Possible Imaging Biomarker for Diagnosis and Progression of the Disease. The Scientific World Journal،Vol. 2012, no. 2012, pp.1-10.
https://search.emarefa.net/detail/BIM-457359
Modern Language Association (MLA)
Hasegawa, Daisuke…[et al.]. Serial MRI Features of Canine GM1 Gangliosidosis : A Possible Imaging Biomarker for Diagnosis and Progression of the Disease. The Scientific World Journal No. 2012 (2012), pp.1-10.
https://search.emarefa.net/detail/BIM-457359
American Medical Association (AMA)
Hasegawa, Daisuke& Yamato, Osamu& Nakamoto, Yuya& Ozawa, Tsuyoshi& Yabuki, Akira& Itamoto, Kazuhito…[et al.]. Serial MRI Features of Canine GM1 Gangliosidosis : A Possible Imaging Biomarker for Diagnosis and Progression of the Disease. The Scientific World Journal. 2012. Vol. 2012, no. 2012, pp.1-10.
https://search.emarefa.net/detail/BIM-457359
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-457359