Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) can be caused by an expanded non-coding hexanucleotide repeat in chromosome 9 open reading frame 72 (C9ORF72), but the underlying mechanism is not known. Suzuki et al. identified the mouse orthologue of C9ORF72 and found that its expression was localized mainly in neurons that, in humans, are known to degenerate in ALS and FTD. These findings suggest that the high expression of C9ORF72 may be responsible for the selective vulnerability of certain neuronal types in ALS and FTD.
References
Suzuki, N. et al. The mouse C9ORF72 ortholog is enriched in neurons known to degenerate in ALS and FTD. Nature Neurosci. http://dx.doi.org/10.1038/nn.3566 (2013)
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Lewis, S. Expression restrictions. Nat Rev Neurosci 14, 821 (2013). https://doi.org/10.1038/nrn3646
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DOI: https://doi.org/10.1038/nrn3646
