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Human Molecular Genetics Advance Access originally published online on December 8, 2005
Human Molecular Genetics 2006 15(2):167-187; doi:10.1093/hmg/ddi446
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© The Author 2005. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Transplanted ALDHhiSSClo neural stem cells generate motor neurons and delay disease progression of nmd mice, an animal model of SMARD1

Stefania Corti1,2, Federica Locatelli1, Dimitra Papadimitriou1, Chiara Donadoni1, Roberto Del Bo1, Marco Crimi1, Andreina Bordoni1, Francesco Fortunato1, Sandra Strazzer3, Giorgia Menozzi3, Sabrina Salani1, Nereo Bresolin1,2,3 and Giacomo P. Comi1,2,*

1Dino Ferrari Centre, Department of Neurological Sciences, University of Milan, IRCCS Foundation Ospedale Maggiore Policlinico, Mangiagalli and Regina Elena, Milan, Italy, 2Centre of Excellence on Neurodegenerative Diseases, University of Milan, Milan, Italy and 3IRCCS Eugenio Medea, Bosisio Parini, Lecco, Italy

* To whom correspondence should be addressed at: Department of Neurological Sciences, University of Milan, IRCCS Foundation Ospedale Maggiore Policlinico, Mangiagalli and Regina Elena, Padiglione Ponti, Via Francesco Sforza 35, 20122 Milan, Italy. Tel: +39 0255033817; fax: +39 0250320430; Email: giacomo.comi{at}unimi.it

Received June 25, 2005; Accepted August 25, 2005

Spinal muscular atrophy with respiratory distress type 1 (SMARD1) is an infantile autosomal-recessive motor neuron disease caused by mutations in the immunoglobulin µ-binding protein 2. We investigated the potential of a spinal cord neural stem cell population isolated on the basis of aldehyde dehydrogenase (ALDH) activity to modify disease progression of nmd mice, an animal model of SMARD1. ALDHhiSSClo stem cells are self-renewing and multipotent and when intrathecally transplanted in nmd mice generate motor neurons properly localized in the spinal cord ventral horns. Transplanted nmd animals presented delayed disease progression, sparing of motor neurons and ventral root axons and increased lifespan. To further investigate the molecular events responsible for these differences, microarray and real-time reverse transcription–polymerase chain reaction analyses of wild-type, mutated and transplanted nmd spinal cord were undertaken. We demonstrated a down-regulation of genes involved in excitatory amino acid toxicity and oxidative stress handling, as well as an up-regulation of genes related to the chromatin organization in nmd compared with wild-type mice, suggesting that they may play a role in SMARD1 pathogenesis. Spinal cord of nmd-transplanted mice expressed high transcript levels for genes related to neurogenesis such as doublecortin (DCX), LIS1 and drebrin. The presence of DCX-expressing cells in adult nmd spinal cord suggests that both exogenous and endogenous neurogeneses may contribute to the observed nmd phenotype amelioration.


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