RNA quality control and protein aggregates in amyotrophic lateral sclerosis: A review

2013 ◽  
Vol 47 (3) ◽  
pp. 330-338 ◽  
Author(s):  
Ashok Verma ◽  
Rup Tandan
Keyword(s):  
Quality Control ◽  
Amyotrophic Lateral Sclerosis ◽  
Protein Aggregates ◽  
Rna Quality ◽  
Rna Quality Control ◽  
Lateral Sclerosis

scholarly journals NFκB is a central regulator of protein quality control in response to protein aggregation stresses via autophagy modulation

2016 ◽  
Vol 27 (11) ◽  
pp. 1712-1727 ◽  
Author(s):  
Mathieu Nivon ◽  
Loïc Fort ◽  
Pascale Muller ◽  
Emma Richet ◽  
Stéphanie Simon ◽  
...  
Keyword(s):  
Quality Control ◽  
Amyotrophic Lateral Sclerosis ◽  
Protein Quality ◽  
Protein Quality Control ◽  
Protein Aggregates ◽  
Familial Amyotrophic Lateral Sclerosis ◽  
Myofibrillar Myopathy ◽  
Conformational Diseases ◽  
Lateral Sclerosis ◽  
Stimulation Of

During cell life, proteins often misfold, depending on particular mutations or environmental changes, which may lead to protein aggregates that are toxic for the cell. Such protein aggregates are the root cause of numerous diseases called “protein conformational diseases,” such as myofibrillar myopathy and familial amyotrophic lateral sclerosis. To fight against aggregates, cells are equipped with protein quality control mechanisms. Here we report that NFκB transcription factor is activated by misincorporation of amino acid analogues into proteins, inhibition of proteasomal activity, expression of the R120G mutated form of HspB5 (associated with myofibrillar myopathy), or expression of the G985R and G93A mutated forms of superoxide dismutase 1 (linked to familial amyotrophic lateral sclerosis). This noncanonical stimulation of NFκB triggers the up-regulation of BAG3 and HspB8 expression, two activators of selective autophagy, which relocalize to protein aggregates. Then NFκB-dependent autophagy allows the clearance of protein aggregates. Thus NFκB appears as a central and major regulator of protein aggregate clearance by modulating autophagic activity. In this context, the pharmacological stimulation of this quality control pathway might represent a valuable strategy for therapies against protein conformational diseases.

microRNA in erythrocytes

2010 ◽  
Vol 38 (1) ◽  
pp. 229-231 ◽  
Author(s):  
Andrew J. Hamilton
Keyword(s):  
Quality Control ◽  
Recent Work ◽  
Total Content ◽  
Human Erythrocytes ◽  
Rna Quality ◽  
Rna Quality Control ◽  
Non Coding Rnas ◽  
Low Levels ◽  
External Signals

Mammalian erythrocytes are generally thought to lack RNA and therefore to be unable to translate new proteins in response to internal or external signals. Support for this long-standing view has accumulated from diverse studies, most of which have focused on the total content of RNA or the overall level of translation. However, more recent work on specific types of RNA has shown the presence in human erythrocytes of both Y RNA and microRNA. The latter seem particularly incongruous given that their normal role is to attenuate the translation of mRNA. Y RNA binds the Ro autoantigen which may have a role in cellular RNA quality control. Therefore the presence of both of these non-coding RNAs indicates the possible existence of other cryptic RNAs in erythrocytes. It also suggests either the existence of low levels of translation or new uncharacterized processes involving microRNA in these cells.

scholarly journals Integrity of SRP RNA is ensured by La and the nuclear RNA quality control machinery

2014 ◽  
Vol 42 (16) ◽  
pp. 10698-10710 ◽  
Author(s):  
Eileen Leung ◽  
Claudia Schneider ◽  
Fu Yan ◽  
Hatem Mohi-El-Din ◽  
Grzegorz Kudla ◽  
...  
Keyword(s):  
Quality Control ◽  
Rna Quality ◽  
Nuclear Rna ◽  
Rna Quality Control ◽  
Srp Rna

scholarly journals Ribosome states signal RNA quality control

2021 ◽  
Vol 81 (7) ◽  
pp. 1372-1383 ◽  
Author(s):  
Karole N. D’Orazio ◽  
Rachel Green
Keyword(s):  
Quality Control ◽  
Rna Quality ◽  
Rna Quality Control

scholarly journals The RNA quality control pathway nonsense-mediated mRNA decay targets cellular and viral RNAs to restrict KSHV

2020 ◽  
Vol 11 (1) ◽  
Author(s):  
Yang Zhao ◽  
Xiang Ye ◽  
Myriam Shehata ◽  
William Dunker ◽  
Zhihang Xie ◽  
...  
Keyword(s):  
Quality Control ◽  
Mrna Decay ◽  
Viral Rnas ◽  
Rna Quality ◽  
Rna Quality Control ◽  
Nonsense Mediated Mrna Decay

NMD-Based Gene Regulation—A Strategy for Fitness Enhancement in Plants?

2019 ◽  
Vol 60 (9) ◽  
pp. 1953-1960 ◽  
Author(s):  
Misato Ohtani ◽  
Andreas Wachter
Keyword(s):  
Quality Control ◽  
Gene Regulation ◽  
Current Knowledge ◽  
Qualitative And Quantitative ◽  
Rna Quality ◽  
Quantitative Level ◽  
Splicing Variants ◽  
Rna Quality Control ◽  
Nonsense Mediated Mrna Decay ◽  
Environmental Responses

Abstract Post-transcriptional RNA quality control is a vital issue for all eukaryotes to secure accurate gene expression, both on a qualitative and quantitative level. Among the different mechanisms, nonsense-mediated mRNA decay (NMD) is an essential surveillance system that triggers degradation of both aberrant and physiological transcripts. By targeting a substantial fraction of all transcripts for degradation, including many alternative splicing variants, NMD has a major impact on shaping transcriptomes. Recent progress on the transcriptome-wide profiling and physiological analyses of NMD-deficient plant mutants revealed crucial roles for NMD in gene regulation and environmental responses. In this review, we will briefly summarize our current knowledge of the recognition and degradation of NMD targets, followed by an account of NMD’s regulation and physiological functions. We will specifically discuss plant-specific aspects of RNA quality control and its functional contribution to the fitness and environmental responses of plants.

scholarly journals The Nuclear Ribonucleoprotein SmD1 Interplays with Splicing, RNA Quality Control, and Posttranscriptional Gene Silencing in Arabidopsis

2016 ◽  
Vol 28 (2) ◽  
pp. 426-438 ◽  
Author(s):  
Emilie Elvira-Matelot ◽  
Florian Bardou ◽  
Federico Ariel ◽  
Vincent Jauvion ◽  
Nathalie Bouteiller ◽  
...  
Keyword(s):  
Quality Control ◽  
Gene Silencing ◽  
Posttranscriptional Gene Silencing ◽  
Rna Quality ◽  
Rna Quality Control ◽  
Nuclear Ribonucleoprotein

Abstract 5538: Development of RNA quality control methods to improve BCR-ABL measurement in whole blood samples

2012 ◽  
Author(s):  
Lauren M. Stanoszek ◽  
Erin L. Crawford ◽  
Thomas Blomquist ◽  
Paige Willey ◽  
James C. Willey
Keyword(s):  
Quality Control ◽  
Whole Blood ◽  
Control Methods ◽  
Blood Samples ◽  
Rna Quality ◽  
Rna Quality Control ◽  
Whole Blood Samples
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