Detection of Relating Molecules for the Pathology of Amyotrophic Lateral Screlosis by the Expressed-Gene Profiling

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The molecular mechanism of the motor neuron death in Amyotrophic lateral sclerosis (ALS) is unknown. We have demonstrated the difference in the expressed-gene profiles between the ALS and spinal anterior horns. We selected 84 clones which had drastic changes changes in their expression levels. Among them, detailed analysis of those matched to ESTs revealed two novel genes (FI39A and FI58G).FI39A cDNA was 4.4kb long and the deduced amino acid sequence should be 838 residues, of which N-terminal half region had ubiquitination-related Ring finger / IBR motif, and bipartite nuclear localization signal (NLS). Northern analysis and ISH showed the expression of FI39A transcript was ubiquitous. The intracellular localization of FI39A protein was perinuclear.FI58G cDNA was 1.8kb long and the deduced amino acid sequence should be 219 residues. The homology search revealed that FI58G protein had no significant homology to any other known molecules. FI58G had a NLS and was localized to the intranuclear region. Northern analysis demonstrated that FI58G mRNA was rich in the brain, spinal cord, heart and muscle.These two novel genes may be involved in the pathogenesis of ALS, although requiring more detailed analysis.