{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,10]],"date-time":"2025-12-10T16:00:35Z","timestamp":1765382435995,"version":"build-2065373602"},"reference-count":88,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2022,12,24]],"date-time":"2022-12-24T00:00:00Z","timestamp":1671840000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"NIH","award":["HL-74185","HL-139047","DK116591","AR-71789"],"award-info":[{"award-number":["HL-74185","HL-139047","DK116591","AR-71789"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Although progressive wasting and weakness of respiratory muscles are the prominent hallmarks of Duchenne muscular dystrophy (DMD) and long-COVID (also referred as the post-acute sequelae of COVID-19 syndrome); however, the underlying mechanism(s) leading to respiratory failure in both conditions remain unclear. We put together the latest relevant literature to further understand the plausible mechanism(s) behind diaphragm malfunctioning in COVID-19 and DMD conditions. Previously, we have shown the role of matrix metalloproteinase-9 (MMP9) in skeletal muscle fibrosis via a substantial increase in the levels of tumor necrosis factor-\u03b1 (TNF-\u03b1) employing a DMD mouse model that was crossed-bred with MMP9-knockout (MMP9-KO or MMP9-\/-) strain. Interestingly, recent observations from clinical studies show a robust increase in neopterin (NPT) levels during COVID-19 which is often observed in patients having DMD. What seems to be common in both (DMD and COVID-19) is the involvement of neopterin (NPT). We know that NPT is generated by activated white blood cells (WBCs) especially the M1 macrophages in response to inducible nitric oxide synthase (iNOS), tetrahydrobiopterin (BH4), and tetrahydrofolate (FH4) pathways, i.e., folate one-carbon metabolism (FOCM) in conjunction with epigenetics underpinning as an immune surveillance protection. Studies from our laboratory, and others researching DMD and the genetically engineered humanized (hACE2) mice that were administered with the spike protein (SP) of SARS-CoV-2 revealed an increase in the levels of NPT, TNF-\u03b1, HDAC, IL-1\u03b2, CD147, and MMP9 in the lung tissue of the animals that were subsequently accompanied by fibrosis of the diaphragm depicting a decreased oscillation phenotype. Therefore, it is of interest to understand how regulatory processes such as epigenetics involvement affect DNMT, HDAC, MTHFS, and iNOS that help generate NPT in the long-COVID patients.<\/jats:p>","DOI":"10.3390\/ijms24010287","type":"journal-article","created":{"date-parts":[[2022,12,27]],"date-time":"2022-12-27T04:35:43Z","timestamp":1672115743000},"page":"287","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["COVID-19 Mimics Pulmonary Dysfunction in Muscular Dystrophy as a Post-Acute Syndrome in Patients"],"prefix":"10.3390","volume":"24","author":[{"given":"Suresh C.","family":"Tyagi","sequence":"first","affiliation":[{"name":"Department of Physiology, School of Medicine, University of Louisville, Louisville, KY 40202, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sathnur","family":"Pushpakumar","sequence":"additional","affiliation":[{"name":"Department of Physiology, School of Medicine, University of Louisville, Louisville, KY 40202, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Utpal","family":"Sen","sequence":"additional","affiliation":[{"name":"Department of Physiology, School of Medicine, University of Louisville, Louisville, KY 40202, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sri Prakash L.","family":"Mokshagundam","sequence":"additional","affiliation":[{"name":"Division of Endocrinology, Metabolism and Diabetes and Robley Rex VA Medical Center, University of Louisville School of Medicine, Louisville, KY 40202, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5254-4067","authenticated-orcid":false,"given":"Dinesh K.","family":"Kalra","sequence":"additional","affiliation":[{"name":"Division of Cardiovascular Medicine, University of Louisville School of Medicine, Louisville, KY 40202, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mohamed A.","family":"Saad","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care and Sleep Disorders Medicine, University of Louisville School of Medicine, Louisville, KY 40202, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2415-3314","authenticated-orcid":false,"given":"Mahavir","family":"Singh","sequence":"additional","affiliation":[{"name":"Department of Physiology, School of Medicine, University of Louisville, Louisville, KY 40202, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,12,24]]},"reference":[{"key":"ref_1","first-page":"1415","article-title":"Musculoskeletal Manifestations of COVID-19: Currently Described Clinical Symptoms and Multimodality Imaging Findings","volume":"42","author":"Omar","year":"2022","journal-title":"Radiogr. A Rev. Publ. Radiol. Soc. N. Am. Inc"},{"key":"ref_2","doi-asserted-by":"crossref","unstructured":"Singh, M., Pushpakumar, S., Bard, N., Zheng, Y., Homme, R.P., Mokshagundam, S.P.L., and Tyagi, S.C. (2022). Simulation of COVID-19 symptoms in a genetically engineered mouse model: Implications for the long haulers. Mol. Cell. Biochem., 1\u201317.","DOI":"10.21203\/rs.3.rs-1236117\/v1"},{"key":"ref_3","doi-asserted-by":"crossref","unstructured":"Homme, R.P., George, A.K., Singh, M., Smolenkova, I., Zheng, Y., Pushpakumar, S., and Tyagi, S.C. (2021). Mechanism of Blood-Heart-Barrier Leakage: Implications for COVID-19 Induced Cardiovascular Injury. Int. J. Mol. Sci., 22.","DOI":"10.3390\/ijms222413546"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1891","DOI":"10.1007\/s11010-021-04054-z","article-title":"Multi-organ damage by COVID-19: Congestive (cardio-pulmonary) heart failure, and blood-heart barrier leakage","volume":"476","author":"Tyagi","year":"2021","journal-title":"Mol. Cell. Biochem."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"607","DOI":"10.1016\/j.nmd.2021.03.011","article-title":"COVID-19 in advanced Duchenne\/Becker muscular dystrophy patients","volume":"31","author":"Levine","year":"2021","journal-title":"Neuromuscul. Disord."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"629549","DOI":"10.3389\/fped.2021.629549","article-title":"Consensus Statement on the Management of Duchenne Muscular Dystrophy in Saudi Arabia during the Coronavirus Disease 2019 Pandemic","volume":"9","author":"Bamaga","year":"2021","journal-title":"Front. Pediatr."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"603","DOI":"10.1016\/j.nmd.2021.04.005","article-title":"Clinical outcome of SARS-CoV-2 infection in 7 adults with Duchenne muscular dystrophy attending a specialist neuromuscular centre","volume":"31","author":"Quinlivan","year":"2021","journal-title":"Neuromuscul. Disord."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1002\/mus.26902","article-title":"The care of patients with Duchenne, Becker, and other muscular dystrophies in the COVID-19 pandemic","volume":"62","author":"Veerapandiyan","year":"2020","journal-title":"Muscle Nerve"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"236","DOI":"10.4103\/0028-3886.43441","article-title":"Duchenne muscular dystrophy","volume":"56","author":"Yiu","year":"2008","journal-title":"Neurol. India"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1038\/s41572-021-00248-3","article-title":"Duchenne muscular dystrophy","volume":"7","author":"Duan","year":"2021","journal-title":"Nat. Rev. Dis. Primers"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"347","DOI":"10.4187\/respcare.09502","article-title":"Improving Access and Guideline Adherence in Pulmonary Care in Patients with Duchenne Muscular Dystrophy","volume":"67","author":"Kaslow","year":"2022","journal-title":"Respir. Care"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"729","DOI":"10.1002\/ppul.25079","article-title":"The effect of emerging molecular and genetic therapies on cardiopulmonary disease in Duchenne muscular dystrophy","volume":"56","author":"McKim","year":"2021","journal-title":"Pediatric Pulmonol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"738","DOI":"10.1002\/ppul.25111","article-title":"Cardiopulmonary phenotypic variability and discordance in Duchenne muscular dystrophy: Implications for new therapies","volume":"56","author":"Birnkrant","year":"2021","journal-title":"Pediatric Pulmonol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1016\/0014-5793(93)81810-M","article-title":"Purification of dystrophin-related protein (utrophin) from lung and its identification in pulmonary artery endothelial cells","volume":"326","author":"Matsumura","year":"1993","journal-title":"FEBS Lett."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1007\/BF02719684","article-title":"Structure and function of the respiratory system of the dystrophic hamster","volume":"168","author":"Schlenker","year":"1990","journal-title":"Lung"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"4439","DOI":"10.1074\/jbc.M805546200","article-title":"Tumor necrosis factor-related weak inducer of apoptosis augments matrix metalloproteinase 9 (MMP-9) production in skeletal muscle through the activation of nuclear factor-kappaB-inducing kinase and p38 mitogen-activated protein kinase: A potential role of MMP-9 in myopathy","volume":"284","author":"Li","year":"2009","journal-title":"J. Biol. Chem."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"2723","DOI":"10.4049\/jimmunol.1101342","article-title":"Osteopontin-stimulated expression of matrix metalloproteinase-9 causes cardiomyopathy in the mdx model of Duchenne muscular dystrophy","volume":"187","author":"Dahiya","year":"2011","journal-title":"J. Immunol. (Baltim. Md. 1950)"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1080\/13813450601118976","article-title":"Lung ischemia-reperfusion injury: Implications of oxidative stress and platelet-arteriolar wall interactions","volume":"113","author":"Ovechkin","year":"2007","journal-title":"Arch. Physiol. Biochem."},{"key":"ref_19","doi-asserted-by":"crossref","unstructured":"Giovarelli, M., Arnaboldi, F., Zecchini, S., Cornaghi, L.B., Nava, A., Sommariva, M., Clementi, E.G.I., and Gagliano, N. (2022). Characterisation of Progressive Skeletal Muscle Fibrosis in the Mdx Mouse Model of Duchenne Muscular Dystrophy: An In Vivo and In Vitro Study. Int. J. Mol. Sci., 23.","DOI":"10.3390\/ijms23158735"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1016\/0304-4165(92)90095-C","article-title":"Turnover rates of different collagen types measured by isotope ratio mass spectrometry","volume":"1156","author":"Rucklidge","year":"1992","journal-title":"Biochim. Et. Biophys. Acta"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"939","DOI":"10.1113\/EP087093","article-title":"The antioxidants neopterin\/7,8-dihydroneopterin: Novel biomarker and muscle protectant in Duchenne muscular dystrophy","volume":"103","author":"Houweling","year":"2018","journal-title":"Exp. Physiol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"995","DOI":"10.1113\/EP087031","article-title":"Neopterin\/7,8-dihydroneopterin is elevated in Duchenne muscular dystrophy patients and protects mdx skeletal muscle function","volume":"103","author":"Lindsay","year":"2018","journal-title":"Exp. Physiol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"709893","DOI":"10.3389\/fcimb.2021.709893","article-title":"Elevated Neopterin Levels Predict Fatal Outcome in SARS-CoV-2-Infected Patients","volume":"11","author":"Chauvin","year":"2021","journal-title":"Front. Cell. Infect. Microbiol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"4161","DOI":"10.1007\/s11010-021-04232-z","article-title":"The potential role of neopterin in COVID-19: A new perspective","volume":"476","author":"Alzahrani","year":"2021","journal-title":"Mol. Cell. Biochem."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"e54721","DOI":"10.15252\/embr.202254721","article-title":"Determinants of epigenetic resistance to HDAC inhibitors in dystrophic fibro-adipogenic progenitors","volume":"23","author":"Consalvi","year":"2022","journal-title":"EMBO Rep."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"560","DOI":"10.1101\/gad.332213.119","article-title":"Loss of an H3K9me anchor rescues laminopathy-linked changes in nuclear organization and muscle function in an Emery-Dreifuss muscular dystrophy model","volume":"34","author":"Harr","year":"2020","journal-title":"Genes Dev."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1402","DOI":"10.1093\/advances\/nmac053","article-title":"Perspectives: On Precision Nutrition Research in Heart, Lung, and Blood Diseases and Sleep Disorders","volume":"13","author":"Pratt","year":"2022","journal-title":"Adv. Nutr. (Bethesda Md.)"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"L239","DOI":"10.1152\/ajplung.00001.2002","article-title":"Induction of oxidative stress and disintegrin metalloproteinase in human heart end-stage failure","volume":"283","author":"Hunt","year":"2002","journal-title":"Am. J. Physiol. Lung Cell. Mol. Physiol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"6349","DOI":"10.1038\/s41598-017-06484-6","article-title":"Toll-like Receptor 4 Deficiency Reduces Oxidative Stress and Macrophage Mediated Inflammation in Hypertensive Kidney","volume":"7","author":"Pushpakumar","year":"2017","journal-title":"Sci. Rep."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"1369","DOI":"10.1139\/cjpp-2016-0744","article-title":"Ablation of toll-like receptor 4 mitigates cardiac mitochondrial dysfunction in hyperhomocysteinemia","volume":"95","author":"Jeremic","year":"2017","journal-title":"Can. J. Physiol. Pharmacol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1007\/s11010-017-3026-9","article-title":"Toll-like receptor 4 mediates vascular remodeling in hyperhomocysteinemia","volume":"433","author":"Familtseva","year":"2017","journal-title":"Mol. Cell. Biochem."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"C596","DOI":"10.1152\/ajpcell.00088.2016","article-title":"Toll-like receptor 4 mutation suppresses hyperhomocysteinemia-induced hypertension","volume":"311","author":"Familtseva","year":"2016","journal-title":"Am. J. Physiol. Cell Physiol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"114","DOI":"10.1016\/j.gene.2019.05.033","article-title":"Exogenous hydrogen sulfide prevents lipopolysaccharide-induced inflammation by blocking the TLR4\/NF-\u03baB pathway in MAC-T cells","volume":"710","author":"Sun","year":"2019","journal-title":"Gene"},{"key":"ref_34","doi-asserted-by":"crossref","unstructured":"Liu, S., Tao, J., Duan, F., Li, H., and Tan, H. (2022). HHcy Induces Pyroptosis and Atherosclerosis via the Lipid Raft-Mediated NOX-ROS-NLRP3 Inflammasome Pathway in apoE-\/- Mice. Cells, 11.","DOI":"10.3390\/cells11152438"},{"key":"ref_35","doi-asserted-by":"crossref","unstructured":"P\u00e9ladeau, C., and Sandhu, J.K. (2021). Aberrant NLRP3 Inflammasome Activation Ignites the Fire of Inflammation in Neuromuscular Diseases. Int. J. Mol. Sci., 22.","DOI":"10.3390\/ijms22116068"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"118069","DOI":"10.1016\/j.lfs.2020.118069","article-title":"Genetic disruption of the inflammasome adaptor ASC has minimal impact on the pathogenesis of Duchenne muscular dystrophy in mdx mice","volume":"257","author":"Lau","year":"2020","journal-title":"Life Sci."},{"key":"ref_37","doi-asserted-by":"crossref","unstructured":"Boursereau, R., Abou-Samra, M., Lecompte, S., Noel, L., and Brichard, S.M. (2018). Downregulation of the NLRP3 inflammasome by adiponectin rescues Duchenne muscular dystrophy. BMC Biol., 16.","DOI":"10.1186\/s12915-018-0501-z"},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"1285","DOI":"10.1038\/cr.2015.139","article-title":"Gasdermin D is an executor of pyroptosis and required for interleukin-1\u03b2 secretion","volume":"25","author":"He","year":"2015","journal-title":"Cell Res."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1038\/nature18629","article-title":"Inflammasome-activated gasdermin D causes pyroptosis by forming membrane pores","volume":"535","author":"Liu","year":"2016","journal-title":"Nature"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"712","DOI":"10.1089\/hum.2016.041","article-title":"Dystrophin Threshold Level Necessary for Normalization of Neuronal Nitric Oxide Synthase, Inducible Nitric Oxide Synthase, and Ryanodine Receptor-Calcium Release Channel Type 1 Nitrosylation in Golden Retriever Muscular Dystrophy Dystrophinopathy","volume":"27","author":"Gentil","year":"2016","journal-title":"Hum. Gene Ther."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"2147","DOI":"10.1093\/hmg\/ddu735","article-title":"Toll-like receptor 4 ablation in mdx mice reveals innate immunity as a therapeutic target in Duchenne muscular dystrophy","volume":"24","author":"Giordano","year":"2015","journal-title":"Hum. Mol. Genet."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1002\/path.4689","article-title":"Divergent impact of Toll-like receptor 2 deficiency on repair mechanisms in healthy muscle versus Duchenne muscular dystrophy","volume":"239","author":"Mojumdar","year":"2016","journal-title":"J. Pathol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"4184","DOI":"10.1038\/s41467-022-31798-z","article-title":"Inactivation of Sirt6 ameliorates muscular dystrophy in mdx mice by releasing suppression of utrophin expression","volume":"13","author":"Georgieva","year":"2022","journal-title":"Nat. Commun."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"608","DOI":"10.1016\/j.jacbts.2021.11.004","article-title":"Cardiovascular Disease in Duchenne Muscular Dystrophy: Overview and Insight Into Novel Therapeutic Targets","volume":"7","author":"Schultz","year":"2022","journal-title":"JACC. Basic Transl. Sci."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"921763","DOI":"10.3389\/fphys.2022.921763","article-title":"Hypoxic Training Ameliorates Skeletal Muscle Microcirculation Vascular Function in a Sirt3-Dependent Manner","volume":"13","author":"Ma","year":"2022","journal-title":"Front. Physiol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"11314","DOI":"10.18632\/aging.103322","article-title":"Lifelong SIRT-1 overexpression attenuates large artery stiffening with advancing age","volume":"12","author":"Machin","year":"2020","journal-title":"Aging"},{"key":"ref_47","doi-asserted-by":"crossref","unstructured":"Acharyya, S., Sharma, S.M., Cheng, A.S., Ladner, K.J., He, W., Kline, W., Wang, H., Ostrowski, M.C., Huang, T.H., and Guttridge, D.C. (2010). TNF inhibits Notch-1 in skeletal muscle cells by Ezh2 and DNA methylation mediated repression: Implications in duchenne muscular dystrophy. PLoS ONE, 5.","DOI":"10.1371\/journal.pone.0012479"},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"537","DOI":"10.1007\/s00401-022-02468-7","article-title":"X-linked myotubular myopathy is associated with epigenetic alterations and is ameliorated by HDAC inhibition","volume":"144","author":"Volpatti","year":"2022","journal-title":"Acta Neuropathol."},{"key":"ref_49","doi-asserted-by":"crossref","unstructured":"Koehorst, E., Odria, R., Cap\u00f3, J., N\u00fa\u00f1ez-Manch\u00f3n, J., Arbex, A., Almendrote, M., Linares-Pardo, I., Natera-de Benito, D., Saez, V., and Nascimento, A. (2022). An Integrative Analysis of DNA Methylation Pattern in Myotonic Dystrophy Type 1 Samples Reveals a Distinct DNA Methylation Profile between Tissues and a Novel Muscle-Associated Epigenetic Dysregulation. Biomedicines, 10.","DOI":"10.3390\/biomedicines10061372"},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1080\/15592294.2018.1445900","article-title":"Developmentally linked human DNA hypermethylation is associated with down-modulation, repression, and upregulation of transcription","volume":"13","author":"Baribault","year":"2018","journal-title":"Epigenetics"},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1139\/cjpp-2020-0093","article-title":"High-methionine diet in skeletal muscle remodeling: Epigenetic mechanism of homocysteine-mediated growth retardation","volume":"99","author":"Singh","year":"2021","journal-title":"Can. J. Physiol. Pharmacol."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"895100","DOI":"10.3389\/fimmu.2022.895100","article-title":"A Barrier to Defend-Models of Pulmonary Barrier to Study Acute Inflammatory Diseases","volume":"13","author":"Herminghaus","year":"2022","journal-title":"Front. Immunol."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"1466","DOI":"10.1164\/rccm.201910-1999PP","article-title":"A Perspective: Division of Lung Diseases at Fifty","volume":"200","author":"Weinmann","year":"2019","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_54","doi-asserted-by":"crossref","unstructured":"Dubuisson, N., Versele, R., Davis-L\u00f3pez de Carrizosa, M.A., Selvais, C.M., Brichard, S.M., and Abou-Samra, M. (2021). Walking down Skeletal Muscle Lane: From Inflammasome to Disease. Cells, 10.","DOI":"10.3390\/cells10113023"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"116654","DOI":"10.1016\/j.lfs.2019.116654","article-title":"Ghrelin improves muscle function in dystrophin-deficient mdx mice by inhibiting NLRP3 inflammasome activation","volume":"232","author":"Chang","year":"2019","journal-title":"Life Sci."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1016\/j.bj.2017.10.002","article-title":"Gasdermin: A new player to the inflammasome game","volume":"40","author":"Morandini","year":"2017","journal-title":"Biomed. J."},{"key":"ref_57","first-page":"2639","article-title":"AIM2-driven inflammasome activation in heart failure","volume":"117","author":"Ruppert","year":"2021","journal-title":"Cardiovasc. Res."},{"key":"ref_58","doi-asserted-by":"crossref","unstructured":"Xu, W., Huang, X., Li, W., Qian, G., Zhou, B., and Wang, X. Carbon monoxide ameliorates LPS-induced acute lung injury via inhibition of alveolar macrophage pyroptosis. Exp. Anim., 2022.","DOI":"10.1538\/expanim.22-0023"},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"916","DOI":"10.1016\/j.ccell.2019.05.002","article-title":"Non-oncogene Addiction to SIRT3 Plays a Critical Role in Lymphomagenesis","volume":"35","author":"Li","year":"2019","journal-title":"Cancer Cell"},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"2990","DOI":"10.1016\/j.bmcl.2013.03.033","article-title":"Identification of novel SIRT3 inhibitor scaffolds by virtual screening","volume":"23","author":"Salo","year":"2013","journal-title":"Bioorg. Med. Chem. Lett."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"627","DOI":"10.1039\/D0CB00216J","article-title":"Mitochondria-targeted inhibitors of the human SIRT3 lysine deacetylase","volume":"2","author":"Troelsen","year":"2021","journal-title":"RSC Chem. Biol."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1002\/biof.1032","article-title":"Sirtuin activators and inhibitors","volume":"38","author":"Villalba","year":"2012","journal-title":"Biofactors"},{"key":"ref_63","first-page":"2047","article-title":"Melatonin Attenuates Anoxia\/Reoxygenation Injury by Inhibiting Excessive Mitophagy through the MT2\/SIRT3\/FoxO3a Signaling Pathway in H9c2 Cells","volume":"14","author":"Wu","year":"2020","journal-title":"Drug Des. Dev."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"8315","DOI":"10.7150\/thno.45922","article-title":"Mitochondrial Sirtuin 3: New emerging biological function and therapeutic target","volume":"10","author":"Zhang","year":"2020","journal-title":"Theranostics"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"L905","DOI":"10.1152\/ajplung.00543.2005","article-title":"3-Deazaadenosine mitigates arterial remodeling and hypertension in hyperhomocysteinemic mice","volume":"291","author":"Ovechkin","year":"2006","journal-title":"Am. J. Physiol. Lung Cell. Mol. Physiol."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"2108","DOI":"10.1002\/jcb.25841","article-title":"Hypermethylation: Causes and Consequences in Skeletal Muscle Myopathy","volume":"118","author":"Majumder","year":"2017","journal-title":"J. Cell. Biochem."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"L301","DOI":"10.1152\/ajplung.00065.2010","article-title":"Functional consequences of the collagen\/elastin switch in vascular remodeling in hyperhomocysteinemic wild-type, eNOS-\/-, and iNOS-\/- mice","volume":"299","author":"Steed","year":"2010","journal-title":"Am. J. Physiol. Lung Cell. Mol. Physiol."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"799","DOI":"10.1089\/152308602760598954","article-title":"Generation of nitrotyrosine precedes activation of metalloproteinase in myocardium of hyperhomocysteinemic rats","volume":"4","author":"Sood","year":"2002","journal-title":"Antioxid. Redox Signal."},{"key":"ref_69","doi-asserted-by":"crossref","unstructured":"Liang, F., Giordano, C., Shang, D., Li, Q., and Petrof, B.J. (2018). The dual CCR2\/CCR5 chemokine receptor antagonist Cenicriviroc reduces macrophage infiltration and disease severity in Duchenne muscular dystrophy (Dmdmdx-4Cv) mice. PLoS ONE, 13.","DOI":"10.1371\/journal.pone.0194421"},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"L223","DOI":"10.1152\/ajplung.00072.2022","article-title":"Circulating extracellular histones exacerbate acute lung injury by augmenting pulmonary endothelial dysfunction via TLR4-dependent mechanism","volume":"323","author":"Kim","year":"2022","journal-title":"Am. J. Physiol. Lung Cell. Mol. Physiol."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"879","DOI":"10.1038\/s41467-022-28531-1","article-title":"TLR4 is a regulator of trained immunity in a murine model of Duchenne muscular dystrophy","volume":"13","author":"Bhattarai","year":"2022","journal-title":"Nat. Commun."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"1874","DOI":"10.1212\/WNL.41.12.1874","article-title":"Long-term benefit from prednisone therapy in Duchenne muscular dystrophy","volume":"41","author":"Fenichel","year":"1991","journal-title":"Neurology"},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1007\/s11940-008-0010-4","article-title":"Treatment options for Duchenne muscular dystrophy","volume":"10","author":"Ciafaloni","year":"2008","journal-title":"Curr. Treat. Opt. Neurol."},{"key":"ref_74","first-page":"149","article-title":"The NorthStar Ambulatory Assessment in Duchenne muscular dystrophy: Considerations for the design of clinical trials","volume":"87","author":"Ricotti","year":"2016","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"667","DOI":"10.1212\/WNL.41.5.667","article-title":"Mononuclear cell analysis of muscle biopsies in prednisone-treated and untreated Duchenne muscular dystrophy. CIDD Study Group","volume":"41","author":"Kissel","year":"1991","journal-title":"Neurology"},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"797","DOI":"10.1089\/hum.2013.092","article-title":"Anti-dystrophin T cell responses in Duchenne muscular dystrophy: Prevalence and a glucocorticoid treatment effect","volume":"24","author":"Flanigan","year":"2013","journal-title":"Hum. Gene Ther."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"6687555","DOI":"10.1155\/2021\/6687555","article-title":"TLR4-NLRP3-GSDMD-Mediated Pyroptosis Plays an Important Role in Aggravated Liver Injury of CD38-\/- Sepsis Mice","volume":"2021","author":"Zhang","year":"2021","journal-title":"J. Immunol. Res."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"603","DOI":"10.3389\/fendo.2019.00603","article-title":"TLR4\/NF-\u03baB Signaling Induces GSDMD-Related Pyroptosis in Tubular Cells in Diabetic Kidney Disease","volume":"10","author":"Wang","year":"2019","journal-title":"Front. Endocrinol."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"660","DOI":"10.1038\/nature15514","article-title":"Cleavage of GSDMD by inflammatory caspases determines pyroptotic cell death","volume":"526","author":"Shi","year":"2015","journal-title":"Nature"},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"1747","DOI":"10.1101\/gad.465908","article-title":"Fibrinogen drives dystrophic muscle fibrosis via a TGFbeta\/alternative macrophage activation pathway","volume":"22","author":"Vidal","year":"2008","journal-title":"Genes Dev."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"645","DOI":"10.1002\/mus.20150","article-title":"Localization and early time course of TGF-beta 1 mRNA expression in dystrophic muscle","volume":"30","author":"Gosselin","year":"2004","journal-title":"Muscle Nerve"},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"653","DOI":"10.1152\/japplphysiol.00248.2021","article-title":"Diaphragm muscle fibrosis involves changes in collagen organization with mechanical implications in Duchenne muscular dystrophy","volume":"132","author":"Sahani","year":"2022","journal-title":"J. Appl. Physiol. (Bethesda Md. 1985)"},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"e15422","DOI":"10.14814\/phy2.15422","article-title":"Hydrogen sulfide mitigates skeletal muscle mitophagy-led tissue remodeling via epigenetic regulation of the gene writer and eraser function","volume":"10","author":"Singh","year":"2022","journal-title":"Physiol. Rep."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1007\/s11010-020-03895-4","article-title":"Epigenetic memory: Gene writer, eraser and homocysteine","volume":"476","author":"Tyagi","year":"2021","journal-title":"Mol. Cell. Biochem."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"583","DOI":"10.1111\/bph.14446","article-title":"Regulators of the transsulfuration pathway","volume":"176","author":"Sbodio","year":"2019","journal-title":"Br. J. Pharmacol."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1172\/JCI118791","article-title":"Defective cystathionine beta-synthase regulation by S-adenosylmethionine in a partially pyridoxine responsive homocystinuria patient","volume":"98","author":"Kluijtmans","year":"1996","journal-title":"J. Clin. Investig."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"2156","DOI":"10.1017\/S0007114513001815","article-title":"Nicotinamide supplementation induces detrimental metabolic and epigenetic changes in developing rats","volume":"110","author":"Li","year":"2013","journal-title":"Br. J. Nutr."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"617953","DOI":"10.3389\/fphys.2020.617953","article-title":"Epigenetics, 1-Carbon Metabolism, and Homocysteine during Dysbiosis","volume":"11","author":"Singh","year":"2020","journal-title":"Front. Physiol."}],"container-title":["International Journal of Molecular Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1422-0067\/24\/1\/287\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T01:49:54Z","timestamp":1760147394000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1422-0067\/24\/1\/287"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,12,24]]},"references-count":88,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2023,1]]}},"alternative-id":["ijms24010287"],"URL":"https:\/\/doi.org\/10.3390\/ijms24010287","relation":{},"ISSN":["1422-0067"],"issn-type":[{"type":"electronic","value":"1422-0067"}],"subject":[],"published":{"date-parts":[[2022,12,24]]}}}