Journal article
A knock-in reporter mouse model for Batten disease reveals predominant expression of Cln3 in visual, limbic and subcortical motor structures
Neurobiology of disease, Vol.41(2), pp.237-248
02/01/2011
DOI: 10.1016/j.nbd.2010.09.011
PMID: 20875858
Abstract
Juvenile neuronal ceroid lipofuscinosis (JNCL) or Batten disease is an autosomal recessive neurodegenerative disorder of children caused by mutation in CLN3. JNCL is characterized by progressive visual impairment, cognitive and motor deficits, seizures and premature death. Information about the localization of CLN3 expressing neurons in the nervous system is limited, especially during development. The present study has systematically mapped the spatial and temporal localization of CLN3 reporter neurons in the entire nervous system including retina, using a knock-in reporter mouse model. CLN3 reporter is expressed predominantly in post-migratory neurons in visual and limbic cortices, anterior and intralaminar thalamic nuclei, amygdala, cerebellum, red nucleus, reticular formation, vestibular nuclei and retina. CLN3 reporter in the nervous system is mainly expressed during the first postnatal month except in the dentate gyrus, parasolitary nucleus and retina, where it is still strongly expressed in adulthood. The predominant distribution of CLN3 reporter neurons in visual, limbic and subcortical motor structures correlates well with the clinical symptoms of JNCL. These findings have also revealed potential target brain regions and time periods for future investigations of the disease mechanisms and therapeutic intervention. (C) 2010 Published by Elsevier Inc.
Details
- Title: Subtitle
- A knock-in reporter mouse model for Batten disease reveals predominant expression of Cln3 in visual, limbic and subcortical motor structures
- Creators
- Song-Lin Ding - Univ Iowa Carver Coll Med, Dept Internal Med, Iowa City, IA 52242 USALuis Tecedor - University of IowaColleen S. Stein - University of IowaBeverly L. Davidson - University of Iowa
- Resource Type
- Journal article
- Publication Details
- Neurobiology of disease, Vol.41(2), pp.237-248
- DOI
- 10.1016/j.nbd.2010.09.011
- PMID
- 20875858
- NLM abbreviation
- Neurobiol Dis
- ISSN
- 0969-9961
- eISSN
- 1095-953X
- Publisher
- Elsevier
- Number of pages
- 12
- Grant note
- Roy J Carver Trust Batten Disease Support and Research Association
- Language
- English
- Date published
- 02/01/2011
- Academic Unit
- Internal Medicine
- Record Identifier
- 9984618639002771
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