<abstract xmlns="http://www.w3.org/1999/xhtml"><p>Neurodegenerative disorders are commonly encountered in medical practices. Such diseases can lead to major morbidity and mortality among the affected individuals. The molecular pathogenesis of these disorders is not yet clear. Recent literature has revealed that mutations in RNA-binding proteins are a key cause of several human neuronal-based diseases. This review discusses the role of RNA metabolism in neurological diseases with specific emphasis on roles of RNA translation and microRNAs in neurodegeneration, RNA-mediated toxicity, repeat expansion diseases and RNA metabolism, molecular pathogenesis of amyotrophic lateral sclerosis and frontotemporal dementia, and neurobiology of survival motor neuron (SMN) and spinal muscular atrophy.</p></abstract>

Neurodegenerative disorders are commonly encountered in medical practices. Such diseases can lead to major morbidity and mortality among the affected individuals. The molecular pathogenesis of these disorders is not yet clear. Recent literature has revealed that mutations in RNA-binding proteins are a key cause of several human neuronal-based diseases. This review discusses the role of RNA metabolism in neurological diseases with specific emphasis on roles of RNA translation and microRNAs in neurodegeneration, RNA-mediated toxicity, repeat expansion diseases and RNA metabolism, molecular pathogenesis of amyotrophic lateral sclerosis and frontotemporal dementia, and neurobiology of survival motor neuron (SMN) and spinal muscular atrophy.

The role of RNA metabolism in neurological diseases

Department of Neurosurgery, University of Calgary, Calgary, Alberta,

Division of Neurosurgery, Department of Surgery, King Saud University, Riyadh,

College of Medicine, Alfaisal University, Riyadh,

Balkan Journal of Medical Genetics

This article is distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

{"article-title":"The role of RNA metabolism in neurological diseases"}

Neurodegenerative disorders are commonly encountered in medical practices. Such diseases can lead to major morbidity and mortality among the affected...

Disease	Cause
Alzheimer’s disease	TDP-43, mir-137/181b, BACE1AS
Down syndrome	TDP-43
Frontotemporal lobar dementia	TDP-43
Amyotrophic lateral sclerosis	TDP-43, mir-206
Familial British dementia	TDP-43
Parkinson’s disease	TDP-43, BC200, SOX20T, mir-34b/c, mir-153
Huntington’s disease	HAR1, DGCR5, TDP-43, FUS/TLS, TUG1, NEAT 1, toxic RNA, mir-132, mir-7, mir-34b
Spinocerebellar ataxia 1, 2, 3, 4	FUS/TLS
Spinocerebellar ataxia 10, 12	Toxic RNA, RAN (repeat associated non ATG translation), expansion in non coding region
Dentatorubral-pallidoluysian atrophy	FUS/TLS
Myopathies	TDP-43
Fragile X tremor ataxia syndrome	Antisense transcription, expansion in non coding region
X-linked mental retardation	UPF3B mutation
Autism	7q22-q33 (non coding RNA)
Spinal muscular atrophy	Mutation in SMN2
Spinocerebellar ataxia	Non coding RNA
Myotonic dystrophy type 1 (DM1)	RAN (repeat associated non ATG translation), expansion in non coding region
Myotonic dystrophy type 2 (DM2)	Expansion in non coding region

The role of RNA metabolism in neurological diseases

Article Category: Review

Published Online: Jul 09, 2016

Page range: 5 - 14

DOI: https://doi.org/10.1515/bjmg-2015-0080

Keywords
Amyotrophic lateral sclerosis, Frontotemporal dementia, RNA, Survival motor neuron (SMN), Spinal muscular atrophy

© 2016 Walter de Gruyter GmbH, Berlin/Boston

This article is distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

RNA role in several neurological diseases.

The role of RNA metabolism in neurological diseases

Article Category: Review

Published Online: Jul 09, 2016

Page range: 5 - 14

DOI: https://doi.org/10.1515/bjmg-2015-0080

KeywordsAmyotrophic lateral sclerosis, Frontotemporal dementia, RNA, Survival motor neuron (SMN), Spinal muscular atrophy

© 2016 Walter de Gruyter GmbH, Berlin/Boston

This article is distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

RNA role in several neurological diseases.

Keywords
Amyotrophic lateral sclerosis, Frontotemporal dementia, RNA, Survival motor neuron (SMN), Spinal muscular atrophy