{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T17:30:02Z","timestamp":1787765402033,"version":"build-2784847793"},"reference-count":70,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2023,12,8]],"date-time":"2023-12-08T00:00:00Z","timestamp":1701993600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000057","name":"National Institute of General Medical Sciences","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000057","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000049","name":"National Institute on Aging","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000049","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Neuroscience Research"],"published-print":{"date-parts":[[2024,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Spinal and bulbar muscular atrophy (SBMA) is an X\u2010linked disorder that affects males who inherit the androgen receptor (AR) gene with an abnormal CAG triplet repeat expansion. The resulting protein contains an elongated polyglutamine (polyQ) tract and causes motor neuron degeneration in an androgen\u2010dependent manner. The precise molecular sequelae of SBMA are unclear. To assist with its investigation and the identification of therapeutic options, we report here a new model of SBMA in\n                    <jats:italic>Drosophila melanogaster<\/jats:italic>\n                    . We generated transgenic flies that express the full\u2010length, human AR with a wild\u2010type or pathogenic polyQ repeat. Each transgene is inserted into the same safe harbor site on the third chromosome of the fly as a single copy and in the same orientation. Expression of pathogenic AR, but not of its wild\u2010type variant, in neurons or muscles leads to consistent, progressive defects in longevity and motility that are concomitant with polyQ\u2010expanded AR protein aggregation and reduced complexity in neuromuscular junctions. Additional assays show adult fly eye abnormalities associated with the pathogenic AR species. The detrimental effects of pathogenic AR are accentuated by feeding flies the androgen, dihydrotestosterone. This new, robust SBMA model can be a valuable tool toward future investigations of this incurable disease.\n                  <\/jats:p>","DOI":"10.1002\/jnr.25278","type":"journal-article","created":{"date-parts":[[2023,12,8]],"date-time":"2023-12-08T03:34:10Z","timestamp":1702006450000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["A phenotypically robust model of spinal and bulbar muscular atrophy in\n                    <i>Drosophila<\/i>"],"prefix":"10.1002","volume":"102","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9146-8017","authenticated-orcid":false,"given":"Kristin","family":"Richardson","sequence":"first","affiliation":[{"name":"Department of Physiology Wayne State University School of Medicine  Detroit Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Medha","family":"Sengupta","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Molecular 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