Visit our Expo - Redox and Inflammation signaling 2012

Session XVII : Cell signaling in health and disease Poster XVII, 22
The Skeletal Muscle Transcriptome In Amyotrophic Lateral Sclerosis.
Benoît HALTER1, Luc DUPUIS1, Marc DE TAPIA1, Franck DI SCALA1, Christa
Niederhause-Wiederkehr2, Philippe Demougin2, Michael Primig2, Jean-Philippe
LOEFFLER1 & Jose-Luis GONZALEZ de AGUILAR1
1Laboratoire de Signalisation Moléculaire et Neurodégénérescence – INSERM U692 -
Faculté de Médecine - 11, rue Humann - 67085 Strasbourg Cedex FRANCE. Tel.: (+33)
390 24 30 8! Fax: (+33) 390 24 30 65. Mail: halter@neurochem.u-strasbg.fr;
gonzalez@neurochem.u-strasbg.fr; loeffler@neurochem.u-strasbg.fr
2Biozentrum and Swiss Institute of Bioinformatics, University of Basel Switzerland
The mechanism underlying ALS pathogenesis is still unclear but growing evidence suggests
that initial alterations in skeletal muscle, preceding the onset of disease symptoms, may
contribute to the disease process. We compared the muscle transcriptome of ALS-related
SOD1(G86R) mice with that of sciatic nerve-axotomized mice. We used a high-density
oligonucleotide microarray and applied advanced bio-informatics protocols to provide a high
throughput and accurate analysis of gene expression on a large scale. An unsupervised
clustering algorithm identified genes that are highly regulated in SOD1(G86R) mice. While
some of these genes represented the characteristic denervation process occurring in ALS,
others were specifically regulated under the pathological condition but not after experimental
denervation. Skeletal muscle provides an additional source of information for the
comprehension of the pathological mechanisms acting in ALS. The systematic approach at
the gene level presented herein may serve to identify new targets for therapeutic intervention
and diagnosis.
Supported by AFM (Association Française contre les Myopathies) and ARS (Association
pour la Recherche sur la Sclérose Latérale Amyotrophique).
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