Altered TDP-43 structure and function: Key insights into aberrant RNA, mitochondrial, and cellular and systemic metabolism in amyotrophic lateral sclerosis

L Jiang, ST Ngo - Metabolites, 2022 - mdpi.com
… , where the clearance of reactive oxygen species was impaired [163,164]. The second … -43
in ALS [165]. The third indication includes altered RNA metabolism in non-coding RNAs such …

Why TDP-43? Why not? Mechanisms of metabolic dysfunction in amyotrophic lateral sclerosis

ML Floare, SP Allen - Neuroscience Insights, 2020 - journals.sagepub.com
… in ALS pathogenesis. As nuclear FUS plays a crucial role in modulating RNA metabolism, …
Moreover, we have recently reported an altered metabolic profile in induced astrocytes from …

RNA deregulation in amyotrophic lateral sclerosis: the noncoding perspective

P Laneve, P Tollis, E Caffarelli - International journal of molecular …, 2021 - mdpi.com
amyotrophic lateral sclerosis (ALS), the most frequent motoneuron (MN) disorder in adults.
Notably, defective RNA metabolism … splicing of mRNAs was altered in FUS-related ALS, with …

RNA modifications and RNA metabolism in neurological disease pathogenesis

B Chatterjee, CKJ Shen, P Majumder - International journal of molecular …, 2021 - mdpi.com
… activate downstream pathways, often altering RNA metabolism. Mutations and changes in …
mutation linked to ALS alters the splicing function of TDP-43, resulting in changed RNAs and …

Alzheimer's, Parkinson's disease and amyotrophic lateral sclerosis gene expression patterns divergence reveals different grade of RNA metabolism involvement

M Garofalo, C Pandini, M Bordoni… - International Journal of …, 2020 - mdpi.com
… pathways of AD, PD, and ALS. Our results pointed out a different degree of RNA metabolism
and dysregulation involvement in AD, PD, and ALS, highlighting a different role for lncRNAs …

Dysregulation of RNA-binding proteins in amyotrophic lateral sclerosis

YC Xue, CS Ng, P Xiang, H Liu, K Zhang… - Frontiers in molecular …, 2020 - frontiersin.org
… /or a gain of toxic function of these ALS-linked RBPs play a … interaction, causing aberrant
RNA metabolism, and by disturbing … and RNA splicing, abnormal RNA transport, altered mRNA …

ALS and FTD: Where RNA metabolism meets protein quality control

J Mandrioli, L Mediani, S Alberti, S Carra - Seminars in cell & …, 2020 - Elsevier
evidence has improved our understanding of the pathomechanisms that lead to amyotrophic
lateral sclerosis (ALS) and … Impaired RNA metabolism, enhanced aggregation of protein-…

RNA is a double-edged sword in ALS pathogenesis

BL Zaepfel, JD Rothstein - Frontiers in cellular neuroscience, 2021 - frontiersin.org
… the roles of altered RNA metabolism and RNA-mediated toxicity … , and C9ORF72 that cause
amyotrophic lateral sclerosis (ALS) and … have on RNA metabolism, as well as how RNA itself …

Globally reduced N6-methyladenosine (m6A) in C9ORF72-ALS/FTD dysregulates RNA metabolism and contributes to neurodegeneration

Y Li, X Dou, J Liu, Y Xiao, Z Zhang, L Hayes, R Wu… - Nature …, 2023 - nature.com
… of RNA metabolism has emerged as key player in multiple neurological disorders 1 . Amyotrophic
lateral sclerosis (ALS… For MeRIP–RT–qPCR, the m 6 A-modified GLuc RNA (NEB) was …

[PDF][PDF] RNA metabolism and DNA damage in neurodegeneration: a new pathological relationship in Amyotrophic Lateral Sclerosis patients

M Garofalo - 2021 - iris.unipv.it
… the hypothesis that RNA dysregulation could be a key contributor to familiar ALS pathogenesis.
… the involvement of an altered RNA metabolism in sporadic ALS cases. Studies based on …