Abstract
RNA metabolism is a vital process through which RNA is produced, transported, regulated, stored, and translated or degraded. Recently, the discoveries of mutations in key RNA binding proteins involved in several human neuronal based diseases have firmly placed the process of RNA metabolism as central to disease etiology. This review first recaps the process of RNA metabolism in the mammalian neuron and describes the roles of RNA granules in this process. Using the recently described alterations in TAR DNA binding protein (TDP-43) and fused in sarcoma/translocated in liposarcoma (FUS/TLS) in amyotrophic lateral sclerosis (ALS) and frontal temporal lobar degeneration (FTLD), we discuss how RNA binding protein abnormalities can affect RNA metabolism. We then discuss two additional RNA based mechanisms distinct from alterations in RNA binding protein function that impact RNA metabolism and result in disease. Cumulatively, these observations provide strong support for the hypothesis that alterations in RNA metabolism can lead to neurodegenerative disease, including ALS.
Keywords: ALS, miRNA, FUS/TLS, TDP-43, myotonic dystrophy, spinal muscular atrophy, RNA metabolism, stress granule, transport granule, Neurodegenerative Disease, frontal temporal lobar degeneration, neuronal intermediate filament inclusion diseas, fragile X tremor ataxia syndrome, spinocerebellar ataxia type 8, oculopharyngeal muscular dystrophy
Current Chemical Biology
Title: RNA Metabolism in Neurodegenerative Disease
Volume: 5 Issue: 2
Author(s): Kathryn Volkening and Michael J. Strong
Affiliation:
Keywords: ALS, miRNA, FUS/TLS, TDP-43, myotonic dystrophy, spinal muscular atrophy, RNA metabolism, stress granule, transport granule, Neurodegenerative Disease, frontal temporal lobar degeneration, neuronal intermediate filament inclusion diseas, fragile X tremor ataxia syndrome, spinocerebellar ataxia type 8, oculopharyngeal muscular dystrophy
Abstract: RNA metabolism is a vital process through which RNA is produced, transported, regulated, stored, and translated or degraded. Recently, the discoveries of mutations in key RNA binding proteins involved in several human neuronal based diseases have firmly placed the process of RNA metabolism as central to disease etiology. This review first recaps the process of RNA metabolism in the mammalian neuron and describes the roles of RNA granules in this process. Using the recently described alterations in TAR DNA binding protein (TDP-43) and fused in sarcoma/translocated in liposarcoma (FUS/TLS) in amyotrophic lateral sclerosis (ALS) and frontal temporal lobar degeneration (FTLD), we discuss how RNA binding protein abnormalities can affect RNA metabolism. We then discuss two additional RNA based mechanisms distinct from alterations in RNA binding protein function that impact RNA metabolism and result in disease. Cumulatively, these observations provide strong support for the hypothesis that alterations in RNA metabolism can lead to neurodegenerative disease, including ALS.
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Cite this article as:
Volkening Kathryn and J. Strong Michael, RNA Metabolism in Neurodegenerative Disease, Current Chemical Biology 2011; 5 (2) . https://dx.doi.org/10.2174/2212796811105020090
DOI https://dx.doi.org/10.2174/2212796811105020090 |
Print ISSN 2212-7968 |
Publisher Name Bentham Science Publisher |
Online ISSN 1872-3136 |
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