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Role of FET proteins in neurodegenerative disorders.
RNA Biol. 2016 Nov; 13(11):1089-1102.RB

Abstract

Neurodegenerative disorders such as Alzheimer disease (AD), frontotemporal dementia (FTD), amyotrophic lateral sclerosis (ALS), Parkinson disease (PD), Huntington's disease (HD), and multiple sclerosis (MS) affect different neuronal cells, and have a variable age of onset, clinical symptoms, and pathological features. Despite the great progress in understanding the etiology of these disorders, the underlying mechanisms remain largely unclear. Among the processes affected in neurodegenerative diseases, alteration in RNA metabolism is emerging as a crucial player. RNA-binding proteins (RBPs) are involved at all stages of RNA metabolism and display a broad range of functions, including modulation of mRNA transcription, splicing, editing, export, stability, translation and localization and miRNA biogenesis, thus enormously impacting regulation of gene expression. On the other hand, aberrant regulation of RBP expression or activity can contribute to disease onset and progression. Recent reports identified mutations causative of neurological disorders in the genes encoding a family of RBPs named FET (FUS/TLS, EWS and TAF15). This review summarizes recent works documenting the involvement of FET proteins in the pathology of ALS, FTLD, essential tremor (ET) and other neurodegenerative diseases. Moreover, clinical implications of recent advances in FET research are critically discussed.

Authors+Show Affiliations

a University of Rome "Foro Italico," , Rome , Italy. b Laboratory of Cellular and Molecular Neurobiology, Fondazione Santa Lucia , Rome , Italy.b Laboratory of Cellular and Molecular Neurobiology, Fondazione Santa Lucia , Rome , Italy.a University of Rome "Foro Italico," , Rome , Italy. b Laboratory of Cellular and Molecular Neurobiology, Fondazione Santa Lucia , Rome , Italy.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

27415968

Citation

Svetoni, Francesca, et al. "Role of FET Proteins in Neurodegenerative Disorders." RNA Biology, vol. 13, no. 11, 2016, pp. 1089-1102.
Svetoni F, Frisone P, Paronetto MP. Role of FET proteins in neurodegenerative disorders. RNA Biol. 2016;13(11):1089-1102.
Svetoni, F., Frisone, P., & Paronetto, M. P. (2016). Role of FET proteins in neurodegenerative disorders. RNA Biology, 13(11), 1089-1102.
Svetoni F, Frisone P, Paronetto MP. Role of FET Proteins in Neurodegenerative Disorders. RNA Biol. 2016;13(11):1089-1102. PubMed PMID: 27415968.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Role of FET proteins in neurodegenerative disorders. AU - Svetoni,Francesca, AU - Frisone,Paola, AU - Paronetto,Maria Paola, Y1 - 2016/07/14/ PY - 2016/10/30/pubmed PY - 2017/5/10/medline PY - 2016/7/15/entrez KW - ALS KW - EWS KW - FUS KW - TAF15 KW - neurodegenerative disorders SP - 1089 EP - 1102 JF - RNA biology JO - RNA Biol VL - 13 IS - 11 N2 - Neurodegenerative disorders such as Alzheimer disease (AD), frontotemporal dementia (FTD), amyotrophic lateral sclerosis (ALS), Parkinson disease (PD), Huntington's disease (HD), and multiple sclerosis (MS) affect different neuronal cells, and have a variable age of onset, clinical symptoms, and pathological features. Despite the great progress in understanding the etiology of these disorders, the underlying mechanisms remain largely unclear. Among the processes affected in neurodegenerative diseases, alteration in RNA metabolism is emerging as a crucial player. RNA-binding proteins (RBPs) are involved at all stages of RNA metabolism and display a broad range of functions, including modulation of mRNA transcription, splicing, editing, export, stability, translation and localization and miRNA biogenesis, thus enormously impacting regulation of gene expression. On the other hand, aberrant regulation of RBP expression or activity can contribute to disease onset and progression. Recent reports identified mutations causative of neurological disorders in the genes encoding a family of RBPs named FET (FUS/TLS, EWS and TAF15). This review summarizes recent works documenting the involvement of FET proteins in the pathology of ALS, FTLD, essential tremor (ET) and other neurodegenerative diseases. Moreover, clinical implications of recent advances in FET research are critically discussed. SN - 1555-8584 UR - https://www.unboundmedicine.com/medline/citation/27415968/Role_of_FET_proteins_in_neurodegenerative_disorders_ DB - PRIME DP - Unbound Medicine ER -