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Large animal models for Batten disease: a review
Krystal Weber1, David A Pearce
1Sanford Children's Health Research Center, Sanford Research/University of South Dakota, Sioux Falls, SD, USA.
Journal of Child Neurology
|September 10, 2013
Summary
Batten disease, a group of inherited childhood disorders, causes severe neurological decline. This review highlights the importance of newly discovered large animal models for advancing Batten disease research.
Area of Science:
- Neuroscience
- Genetics
- Pediatric Neurology
Background:
- Neuronal ceroid lipofuscinoses, or Batten disease, are inherited childhood neurodegenerative disorders.
- These conditions lead to progressive vision loss, motor and cognitive decline, and premature death.
- Over 10 genes are linked to various forms of Batten disease.
Purpose of the Study:
- To review and discuss the significance of large animal models in Batten disease research.
- To highlight the advantages of large animal models over traditional mouse models.
Main Methods:
- Literature review of existing research on Batten disease.
- Analysis of studies utilizing large animal models for Batten disease.
Main Results:
- Several naturally occurring large animal models for Batten disease have been identified.
- These models offer unique opportunities for studying disease mechanisms and testing therapies.
Conclusions:
- Large animal models are crucial for advancing our understanding and treatment of Batten disease.
- Further research using these models is essential for developing effective interventions.