{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,14]],"date-time":"2026-05-14T01:01:58Z","timestamp":1778720518073,"version":"3.51.4"},"reference-count":29,"publisher":"MDPI AG","issue":"22","license":[{"start":{"date-parts":[[2023,11,11]],"date-time":"2023-11-11T00:00:00Z","timestamp":1699660800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"National Institutes of Health (NIH)\/National Institute of Neurological Disorders and Stroke","award":["NS089701"],"award-info":[{"award-number":["NS089701"]}]},{"name":"National Institutes of Health (NIH)\/National Institute of Neurological Disorders and Stroke","award":["NS095962"],"award-info":[{"award-number":["NS095962"]}]},{"name":"National Institutes of Health (NIH)\/National Institute of Neurological Disorders and Stroke","award":["NS110455"],"award-info":[{"award-number":["NS110455"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>A dominant mutation in hnRNPA1 causes amyotrophic lateral sclerosis (ALS), but it is not known whether this mutation leads to motor neuron death through increased or decreased function. To elucidate the relationship between pathogenic hnRNPA1 mutation and its native function, we created novel transgenic rats that overexpressed wildtype rat hnRNPA1 exclusively in motor neurons. This targeted expression of wildtype hnRNPA1 caused severe motor neuron loss and subsequent denervation muscle atrophy in transgenic rats that recapitulated the characteristics of ALS. These findings demonstrate that the augmentation of hnRNPA1 expression suffices to trigger motor neuron degeneration and the manifestation of ALS-like phenotypes. It is reasonable to infer that an amplification of an as-yet undetermined hnRNPA1 function plays a pivotal role in the pathogenesis of familial ALS caused by pathogenic hnRNPA1 mutation.<\/jats:p>","DOI":"10.3390\/ijms242216214","type":"journal-article","created":{"date-parts":[[2023,11,13]],"date-time":"2023-11-13T02:02:42Z","timestamp":1699840962000},"page":"16214","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Increase in hnRNPA1 Expression Suffices to Kill Motor Neurons in Transgenic Rats"],"prefix":"10.3390","volume":"24","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3028-1945","authenticated-orcid":false,"given":"Bo","family":"Huang","sequence":"first","affiliation":[{"name":"Department of Pathology, Thomas Jefferson University, 1020 Locust Street, Philadelphia, PA 19107, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2633-7096","authenticated-orcid":false,"given":"Xionghao","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Pathology, Thomas Jefferson University, 1020 Locust Street, Philadelphia, PA 19107, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tingting","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Environmental Health Sciences, Robert Stempel College of Public Health & Social Work, The Center for Translational Sciences, Florida International University, Port St. Lucie, FL 34987, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Qinxue","family":"Wu","sequence":"additional","affiliation":[{"name":"Department of Pathology, Thomas Jefferson University, 1020 Locust Street, Philadelphia, PA 19107, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7033-2498","authenticated-orcid":false,"given":"Cao","family":"Huang","sequence":"additional","affiliation":[{"name":"Department of Pathology, Thomas Jefferson University, 1020 Locust Street, Philadelphia, PA 19107, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xu-Gang","family":"Xia","sequence":"additional","affiliation":[{"name":"Department of Environmental Health Sciences, Robert Stempel College of Public Health & Social Work, The Center for Translational Sciences, Florida International University, Port St. Lucie, FL 34987, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hongxia","family":"Zhou","sequence":"additional","affiliation":[{"name":"Department of Environmental Health Sciences, Robert Stempel College of Public Health & Social Work, The Center for Translational Sciences, Florida International University, Port St. Lucie, FL 34987, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2023,11,11]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1186\/s40478-022-01433-5","article-title":"Protein citrullination marks myelin protein aggregation and disease progression in mouse ALS models","volume":"10","author":"Yusuf","year":"2022","journal-title":"Acta Neuropathol. 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