ArticleTheScientificWorldJournal2012
Serial MRI features of canine GM1 gangliosidosis: a possible imaging biomarker for diagnosis and progression of the disease.
Article in TheScientificWorldJournal, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed, 16 citations in OpenAlex.
- Magnetic resonance imaging pattern recognition of metabolic and neurodegenerative encephalopathies in dogs and cats.Frontiers in veterinary science · 2024Article
- Case report: Recovery and sequential imaging of a patient with osmotic demyelination syndrome.Frontiers in veterinary science · 2023Article
- A hypomyelinating leukodystrophy in German Shepherd dogs.Journal of veterinary internal medicine · 2021Article
- Positioning Head Tilt in Canine Lysosomal Storage Disease: A Retrospective Observational Descriptive Study.Frontiers in veterinary science · 2021Article
- 7T MRI Predicts Amelioration of Neurodegeneration in the Brain after AAV Gene Therapy.Molecular therapy. Methods & clinical development · 2020Article
- Cognitive Abilities of Dogs with Mucopolysaccharidosis I: Learning and Memory.Animals : an open access journal from MDPI · 2020Article
- Characterization of Structural Connectivity of the Default Mode Network in Dogs using Diffusion Tensor Imaging.Scientific reports · 2016Article
- GM1 gangliosidosis in a Japanese domestic cat: a new variant identified in Hokkaido, Japan.The Journal of veterinary medical science · 2016Article
- Molecular epidemiology of canine GM1 gangliosidosis in the Shiba Inu breed in Japan: relationship between regional prevalence and carrier frequency.BMC veterinary research · 2013Article
- Magnetic resonance findings of the corpus callosum in canine and feline lysosomal storage diseases.PloS one · 2013Article
- Neuronal ceroid lipofuscinosis in Border Collie dogs in Japan: clinical and molecular epidemiological study (2000-2011).TheScientificWorldJournal · 2012Article
- A digital atlas of the dog brain.PloS one · 2012Article
Corrections and comments
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Authors and funding
11 authors at 4 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
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.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.