Journal
HUMAN MOLECULAR GENETICS
Volume 19, Issue -, Pages R46-R64Publisher
OXFORD UNIV PRESS
DOI: 10.1093/hmg/ddq137
Keywords
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Funding
- Amytrophic Lateral Sclerosis Association
- Ludwig Institute for Cancer Research
- Human Frontier Science Program
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Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are neurodegenerative diseases with clinical and pathological overlap. Landmark discoveries of mutations in the transactive response DNA-binding protein (TDP-43) and fused in sarcoma/translocated in liposarcoma (FUS/TLS) as causative of ALS and FTLD, combined with the abnormal aggregation of these proteins, have initiated a shifting paradigm for the underlying pathogenesis of multiple neurodegenerative diseases. TDP-43 and FUS/TLS are both RNA/DNA-binding proteins with striking structural and functional similarities. Their association with ALS and other neurodegenerative diseases is redirecting research efforts toward understanding the role of RNA processing regulation in neurodegeneration.
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