Matrin 3 in neuromuscular disease: physiology and pathophysiology

Malik, Aatika; Barmada, Sami J.

doi:10.1172/jci.insight.143948

Cited by 38 publications

(29 citation statements)

References 126 publications

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“…In HIV-infected cells, Sarracino et al [35] found MATR3 to be essential for RNA processing. MATR3 phoshorylation was found to greatly enhance its DNA binding ability [36, 37]. It is well documented its implications in Amyotrophic lateral sclerosis [38], a disease causing muscle weakness and respiratory failures, symptoms common in Covid-19 patients.…”

Section: Resultsmentioning

confidence: 99%

CIGB-300 synthetic peptide, an antagonist of CK2 kinase activity, as a treatment for Covid-19. A computational biology approach

Miranda

Bringas

Fernández-de-Cossío

et al. 2021

Preprint

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Drug repositioning became the first choice for treating Covid-19 patients due to the urgent need to deal with the pandemic. Similarities in the hijacking mechanisms used by SARS-CoV-2 and several type of cancer, suggest the repurposing of cancer drugs to treat Covid-19. CK2 kinase antagonists have been proposed for the treatment of cancer. A recent study in cells infected with SARS-CoV-2 virus found a significant CK2 kinase activity, and the use of a CK2 inhibitor showed antiviral responses. CIGB-300, originally designed as an anticancer peptide, is an antagonist of CK2 kinase activity that binds to CK2 phospho-acceptor sites. Recent preliminary results show an antiviral activity of CIGB-300 versus a surrogate model of coronavirus. Here we present a computational biology study that provides evidences at the molecular level of how CIGB-300 might interfere with SARS-CoV-2 life cycle inside infected human cells. First, from SARS-CoV studies, we infer the potential incidence of CIGB-300 in SARS-CoV-2 interference on immune response. Next, from the analysis of multiple Omics data, we propose the action of CIGB-300 since early stage of viral infections perturbing the virus hijacking of RNA splicing machinery. It was also predicted the interference of CIGB-300 in virus-host interactions responsible for the high infectivity and the particular immune response to SARS-CoV-2 infection. Further, we provide evidences of CIGB-300 attenuation of phenotypes related to muscle, bleeding, coagulation and respiratory disorders.

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Section: Resultsmentioning

confidence: 99%

CIGB-300 synthetic peptide, an antagonist of CK2 kinase activity, as a treatment for Covid-19. A computational biology approach

Miranda

Bringas

Fernández-de-Cossío

et al. 2021

Preprint

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“…MATR3 Mutations Matrin 3 (MATR3) is a highly conserved RNA binding protein [390]. Three mutations of this protein have been identified (F115C, Y622A, P154S, and S85C) to be involved in both familial and sporadic ALS [380,391]. A transgenic mouse carrying the human WT MATR3 was generated and developed either hindlimb paralysis or hindlimb and forelimb muscle atrophy [392].…”

Section: Als-related Protein Mutations For Novel or Potential Animal Modelsmentioning

confidence: 99%

Nearly 30 Years of Animal Models to Study Amyotrophic Lateral Sclerosis: A Historical Overview and Future Perspectives

Bonifacino

Zerbo

Balbi

et al. 2021

IJMS

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Amyotrophic lateral sclerosis (ALS) is a fatal, multigenic, multifactorial, and non-cell autonomous neurodegenerative disease characterized by upper and lower motor neuron loss. Several genetic mutations lead to ALS development and many emerging gene mutations have been discovered in recent years. Over the decades since 1990, several animal models have been generated to study ALS pathology including both vertebrates and invertebrates such as yeast, worms, flies, zebrafish, mice, rats, guinea pigs, dogs, and non-human primates. Although these models show different peculiarities, they are all useful and complementary to dissect the pathological mechanisms at the basis of motor neuron degeneration and ALS progression, thus contributing to the development of new promising therapeutics. In this review, we describe the up to date and available ALS genetic animal models, classified by the different genetic mutations and divided per species, pointing out their features in modeling, the onset and progression of the pathology, as well as their specific pathological hallmarks. Moreover, we highlight similarities, differences, advantages, and limitations, aimed at helping the researcher to select the most appropriate experimental animal model, when designing a preclinical ALS study.

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“…PABPN1 also functions with other proteins in regenerating skeletal muscle and in differentiating C2C12 cells. In particular, it interacts with matrin3, an RNA- and DNA-binding nuclear matrix protein whose mutations are associated with a rare late-onset distal myopathy and ALS ( Iradi et al, 2018 ; Malik and Barmada, 2021 ). The two proteins colocalize in discrete nuclear foci and promote myogenesis by regulating polyadenylation site selection in myogenic transcripts ( Banerjee et al, 2017 ).…”

Section: Rna-binding Proteins Involved In Skeletal Muscle Development Regeneration and Diseasementioning

confidence: 99%

“…Thus it is important to gain an overall picture of post-transcriptional circuits exerted by RBPs in muscle physiopathology. The implication of several best-characterized RBPs in muscle development and neuromuscular disease has been discussed in more detail ( Apponi et al, 2011 ; Dasgupta and Ladd, 2012 ; Raz and Raz, 2014 ; Hinkle et al, 2019 ; Nikonova et al, 2019 ; Sznajder and Swanson, 2019 ; López-Martínez et al, 2020 ; Malik and Barmada, 2021 ). This review attempts to provide a comprehensive outline of studies in understanding functional roles of RBPs during vertebrate embryonic myogenesis and adult skeletal muscle regeneration, with the aim to identify the unanswered questions that merit further investigations.…”

Section: Introductionmentioning

confidence: 99%

RNA-Binding Proteins in the Post-transcriptional Control of Skeletal Muscle Development, Regeneration and Disease

Shi

Grifone

2021

Front. Cell Dev. Biol.

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Embryonic myogenesis is a temporally and spatially regulated process that generates skeletal muscle of the trunk and limbs. During this process, mononucleated myoblasts derived from myogenic progenitor cells within the somites undergo proliferation, migration and differentiation to elongate and fuse into multinucleated functional myofibers. Skeletal muscle is the most abundant tissue of the body and has the remarkable ability to self-repair by re-activating the myogenic program in muscle stem cells, known as satellite cells. Post-transcriptional regulation of gene expression mediated by RNA-binding proteins is critically required for muscle development during embryogenesis and for muscle homeostasis in the adult. Differential subcellular localization and activity of RNA-binding proteins orchestrates target gene expression at multiple levels to regulate different steps of myogenesis. Dysfunctions of these post-transcriptional regulators impair muscle development and homeostasis, but also cause defects in motor neurons or the neuromuscular junction, resulting in muscle degeneration and neuromuscular disease. Many RNA-binding proteins, such as members of the muscle blind-like (MBNL) and CUG-BP and ETR-3-like factors (CELF) families, display both overlapping and distinct targets in muscle cells. Thus they function either cooperatively or antagonistically to coordinate myoblast proliferation and differentiation. Evidence is accumulating that the dynamic interplay of their regulatory activity may control the progression of myogenic program as well as stem cell quiescence and activation. Moreover, the role of RNA-binding proteins that regulate post-transcriptional modification in the myogenic program is far less understood as compared with transcription factors involved in myogenic specification and differentiation. Here we review past achievements and recent advances in understanding the functions of RNA-binding proteins during skeletal muscle development, regeneration and disease, with the aim to identify the fundamental questions that are still open for further investigations.

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Matrin 3 in neuromuscular disease: physiology and pathophysiology

Cited by 38 publications

References 126 publications

CIGB-300 synthetic peptide, an antagonist of CK2 kinase activity, as a treatment for Covid-19. A computational biology approach

CIGB-300 synthetic peptide, an antagonist of CK2 kinase activity, as a treatment for Covid-19. A computational biology approach

Nearly 30 Years of Animal Models to Study Amyotrophic Lateral Sclerosis: A Historical Overview and Future Perspectives

RNA-Binding Proteins in the Post-transcriptional Control of Skeletal Muscle Development, Regeneration and Disease

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