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Publication : RNA binding proteins and the pathological cascade in ALS/FTD neurodegeneration.

First Author  Ito D Year  2017
Journal  Sci Transl Med Volume  9
Issue  415 PubMed ID  29118263
Mgi Jnum  J:249958 Mgi Id  MGI:5921413
Doi  10.1126/scitranslmed.aah5436 Citation  Ito D, et al. (2017) RNA binding proteins and the pathological cascade in ALS/FTD neurodegeneration. Sci Transl Med 9(415)
abstractText  Advanced genetic approaches have accelerated the identification of causative genes linked to the neurodegenerative diseases amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Most of the disease-related proteins encoded by these genes form aggregates in the cellular machineries that regulate RNA and protein quality control in cells. Cross-talk among the signaling pathways governing these machineries leads to pathological cascades mediated by the accumulation of mutant RNA binding proteins. We outline the molecular basis of ALS and FTD pathogenesis and discuss the prospects for therapeutic strategies to treat these diseases.
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