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doi: 10.1242/10.1242/jcs.00169


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Journal of Cell Science 115, 4735-4741 (2002)
doi: 10.1242/jcs.00169


Research Article

Absence of retinoids can induce motoneuron disease in the adult rat and a retinoid defect is present in motoneuron disease patients

Jonathan Corcoran*,{ddagger}, Po Lin So and Malcolm Maden

MRC Centre for Developmental Neurobiology, New Hunt's House, King's College London, Guy's Campus, London SE1 1UL, UK
* Present address: Wolfson Centre for Age-Related Diseases, King's College London, Guy's Campus, London SE1 1UL, UK

{ddagger} Author for correspondence (e-mail: jonathan.corcoran{at}kcl.ac.uk)

Accepted 12 September 2002

We generated retinoid-deficient adult rats by the removal of retinoids from their diet. We show that their motoneurons undergo neurodegeneration and that there is an accumulation of neurofilaments and an increase in astrocytosis, which is associated with motoneuron disease. These effects are mediated through the retinoic acid receptor {alpha}. The same receptor deficit is found in motoneurons from patients suffering from spontaneous amyotrophic lateral sclerosis. Furthermore, we show that there is a loss of expression of the retinaldehyde dehydrogenase enzyme II in motoneurons. Therefore, we propose that a defect in the retinoid signalling pathway is in part be responsible for some types of motoneuron disease.

Key words: Motoneuron, Retinoic acid, Neurodegeneration


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© The Company of Biologists Ltd 2002