ArticleBiomolecules2026
Absence of Neuromuscular Dysfunction in Mice with Gut Epithelium-Restricted Expression of ALS Mutation hSOD1
Li Dong, Xuejun Li, Ang Li, Jianxun Yi, Yanan Vockery, Yan Chang, Zui Pan, Marco Brotto, Jingsong Zhou
Abstract read
In one paragraphArticle in Biomolecules, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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5 · Who and what moneyAuthors and funding
9 authors.
Li DongDepartment of Kinesiology, College of Nursing and Health Innovation, University of Texas at Arlington, Arlington, TX 76019, USA.ORCID 0009-0005-1574-068X Xuejun LiDepartment of Kinesiology, College of Nursing and Health Innovation, University of Texas at Arlington, Arlington, TX 76019, USA.
Ang LiDepartment of Kinesiology, College of Nursing and Health Innovation, University of Texas at Arlington, Arlington, TX 76019, USA.ORCID 0000-0002-8784-4702 Jianxun YiDepartment of Kinesiology, College of Nursing and Health Innovation, University of Texas at Arlington, Arlington, TX 76019, USA.
Yanan VockeryDepartment of Kinesiology, College of Nursing and Health Innovation, University of Texas at Arlington, Arlington, TX 76019, USA.
Yan ChangBone-Muscle Research Center, College of Nursing and Health Innovation, University of Texas at Arlington, Arlington, TX 76010, USA.
Zui PanBone-Muscle Research Center, College of Nursing and Health Innovation, University of Texas at Arlington, Arlington, TX 76010, USA.ORCID 0000-0003-4105-901X Marco BrottoBone-Muscle Research Center, College of Nursing and Health Innovation, University of Texas at Arlington, Arlington, TX 76010, USA.
Jingsong ZhouDepartment of Kinesiology, College of Nursing and Health Innovation, University of Texas at Arlington, Arlington, TX 76019, USA.
Funding
Preserving Mitochondrial Function for Alleviating ALS ProgressionR01NS105621 · NINDS · UNIVERSITY OF TEXAS ARLINGTON · PI BROTTO, MARCO, ZHOU, JINGSONG · 2019 to 2023
$2.9MCa signaling cross-talk from SR to mitochondria in heart muscleR01HL138570 · NHLBI · OHIO STATE UNIVERSITY · PI LIN, PEI-HUI, ZHOU, JINGSONG · 2018 to 2021
$2.6MMultifaceted role of MG53 in alleviating neuromuscular function decline in ALSR01NS129219 · NINDS · UNIVERSITY OF TEXAS ARLINGTON · PI Jianjie Ma, Lyle Wilfred Ostrow · 2023 to 2026
$2.5MCa Signaling in Progression of Amyotrophic Lateral Sclerosis in Skeletal MuscleR01AR057404 · NIAMS · RUSH UNIVERSITY MEDICAL CENTER · PI ZHOU, JINGSONG · 2010 to 2014
$1.6MBank of America 16-IIP-288National Institute of Health R01HL138570National Institute of Health R01NS105621NHLBI NIH HHS R01 HL138570NIAMS NIH HHS R01 AR057404NINDS NIH HHS R01 NS105621NINDS NIH HHS R01 NS129219United States Department of Defense AL170061(W81XWH1810684)
6 · The paper itselfAbstract
Amyotrophic Lateral Sclerosis (ALS) is a devastating neuromuscular disorder characterized by the progressive loss of motor neurons and skeletal muscle, ultimately leading to respiratory failure and death, typically within 3-5 years following diagnosis. While the death of motor neurons is the pathological hallmark, ALS is increasingly recognized as a systemic disorder involving non-motor systems. Gastrointestinal dysfunction has been widely observed in both ALS patients and animal models. However, because gut abnormalities and neuromuscular degeneration are intertwined during ALS disease progression, it remains unclear whether these gut abnormalities are merely a consequence of neuromuscular degeneration or whether they play a crucial role in initiating it. In this study, we investigated whether an ALS-associated mutation expressed exclusively in the gut can directly affect neuromuscular function. We generated a novel transgenic mouse model, Gut-hG93A, which overexpresses the human ALS mutation hSOD1
Indexed as
Amyotrophic Lateral SclerosisIntestinal MucosaMutationSuperoxide Dismutase-1AnimalsDisease Models, AnimalHumansIntestinal Barrier FunctionMiceMice, TransgenicMuscle, SkeletalSOD1 protein, humanSuperoxide Dismutase-1amyotrophic lateral sclerosis (ALS)Cre-Loxgut epitheliumhuman ALS mutation hSOD1G93Aintestinal permeabilitymuscle contractilityskeletal muscletransgenic mouse
Identifiers
PMID41750323
PMCPMC12938467
What Socratic holds
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