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A major set of gene regulations in G86R muscles resembled those of surgically denervated muscles, but many others appeared specific to the ALS condition. The first significant transcriptional changes appeared in a subpopulation of mice before the onset of overt clinical symptoms and motor neuron death. These early changes affected genes involved in detoxification (e.g., ALDH3, metallothionein-2, and thioredoxin-1) and regeneration (e.g., BTG1, RB1, and RUNX1) but also tissue degradation (e.g., C\/EBP\u03b4 and DDIT4) and cell death (e.g., ankyrin repeat domain-1, CDKN1A, GADD45\u03b1, and PEG3). Of particular interest, metallothionein-1 and -2, ATF3, cathepsin-Z, and galectin-3 genes appeared, among others, commonly regulated in both skeletal muscle (our present data) and spinal motor neurons (as previously reported) of paralyzed ALS mice. The importance of these findings is twofold. First, they designate the distal part of the motor unit as a primary site of disease. Second, they identify specific gene regulations to be explored in the search for therapeutic strategies that could alleviate disease before motor neuron death manifests clinically. <\/jats:p>","DOI":"10.1152\/physiolgenomics.00017.2007","type":"journal-article","created":{"date-parts":[[2007,11,13]],"date-time":"2007-11-13T21:06:07Z","timestamp":1194987967000},"page":"207-218","source":"Crossref","is-referenced-by-count":125,"title":["Gene profiling of skeletal muscle in an amyotrophic lateral sclerosis mouse model"],"prefix":"10.1152","volume":"32","author":[{"given":"Jose-Luis","family":"Gonzalez de Aguilar","sequence":"first","affiliation":[{"name":"Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale, U692, Laboratoire de Signalisations Mol\u00e9culaires et Neurod\u00e9g\u00e9n\u00e9rescence, Strasbourg"},{"name":"Universit\u00e9 Louis Pasteur, Facult\u00e9 de M\u00e9decine, Unit\u00e9 Mixte de Recherche en Sant\u00e9-692, Strasbourg, 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