Journal
NEURON
Volume 79, Issue 3, Pages 416-438Publisher
CELL PRESS
DOI: 10.1016/j.neuron.2013.07.033
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Funding
- NIH [R01-NS27036, K99-NS075216]
- Ludwig Institute for Cancer Research
- National Institute of Aging [T32 AG 000216]
- international Human Frontier Science Program Organization
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Breakthrough discoveries identifying common genetic causes for amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) have transformed our view of these disorders. They share unexpectedly similar signatures, including dysregulation in common molecular players including TDP-43, FUS/TLS, ubiquilin-2, VCP, and expanded hexanucleotide repeats within the C9ORF72 gene. Dysfunction in RNA processing and protein homeostasis is an emerging theme. We present the case here that these two processes are intimately linked, with disease-initiated perturbation of either leading to further deviation of both protein and RNA homeostasis through a feedforward loop including cell-to-cell prion-like spread that may represent the mechanism for relentless disease progression.
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