{"doi":"10.1016/j.nbd.2008.12.009","title":"Recovery of function in a myogenic mouse model of spinal bulbar muscular atrophy","abstract":null,"journal":"Neurobiology of Disease","year":2009,"id":663026,"datarank":0.586803450814222,"base_score":3.912023005428146,"endowment":3.912023005428146,"self_citation_contribution":0.586803450814222,"citation_network_contribution":0.0,"self_endowment_contribution":0.586803450814222,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":49,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":null,"is_data_producer":false,"deposit_databanks":null,"is_oa":false,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":null,"fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":163064,"name":"Zhigang Yu","orcid":null,"position":1,"is_corresponding":false},{"id":1730979,"name":"Kaiguo Mo","orcid":null,"position":2,"is_corresponding":false},{"id":1730980,"name":"D. Ashley Monks","orcid":null,"position":3,"is_corresponding":false},{"id":331941,"name":"Andrew P. Lieberman","orcid":"0000-0001-9032-580X","position":4,"is_corresponding":false},{"id":499445,"name":"S. Marc Breedlove","orcid":"0000-0003-4359-4315","position":5,"is_corresponding":false},{"id":324715,"name":"Cynthia L. Jordan","orcid":null,"position":6,"is_corresponding":false},{"id":1730978,"name":"Jamie A. Johansen","orcid":null,"position":0,"is_corresponding":false}],"reference_count":0,"raw_metadata":{"has_enrichment":true,"resolved":true,"title":"Recovery of function in a myogenic mouse model of spinal bulbar muscular atrophy","abstract":"With this paper, we deliberately challenge the prevailing neurocentric theory of the etiology of spinal bulbar muscular atrophy (SBMA). We offer data supporting an alternative view that androgen receptor (AR) acts in skeletal muscles to cause the symptoms of SBMA. While SBMA has been linked to a CAG repeat expansion in the AR gene and mutant AR is presumed to act in motoneurons to cause SBMA, we find that over-expression of wild type AR solely in skeletal muscle fibers results in the same androgen-dependent disease phenotype as when mutant AR is broadly expressed. Like other recent SBMA mouse models, transgenic (tg) females in our model exhibit a motor phenotype only when exposed to androgens, and this motor dysfunction is independent of motoneuronal or muscle fiber cell death. Muscles from symptomatic females also show denervation-like changes in gene expression comparable to a knock-in model of SBMA. Furthermore, once androgen treatment ends, tg females rapidly recover motor function and muscle gene expression, demonstrating the strict androgen-dependence of the disease phenotype in our model. Our results argue that SBMA may be caused by AR acting in muscle.","is_dataset_classified":null,"base_score":3.912023005428146,"endowment":3.912023005428146,"datacite_reuse_total":0,"file_count":0,"downloads":0,"views":0,"has_version_chain":false,"is_dataset":false,"is_oa":false,"pmid":"19211034","pmcid":"PMC4209964","openalex_id":"https://openalex.org/W1973538757","authors":[],"funders":[{"funder_name":"National Institutes of Health","grant_id":"NS045195","title":null},{"funder_name":"National Institutes of Health","grant_id":"NS055746","title":null},{"funder_name":"National Institutes of Health","grant_id":"NS51257","title":null},{"funder_name":"National Institutes of Health","grant_id":"NS54517","title":null},{"funder_name":"NINDS NIH HHS","grant_id":"R01 NS051257","title":null},{"funder_name":"NINDS NIH HHS","grant_id":"F31 NS054517","title":null},{"funder_name":"NIMH NIH HHS","grant_id":"T32 MH070343","title":null},{"funder_name":"NINDS NIH HHS","grant_id":"R01 NS045195","title":null},{"funder_name":"NINDS NIH HHS","grant_id":"R01 NS055746","title":null}],"total_grants":9,"fwci":1.6212,"citation_percentile":0.8013539,"influential_citations":0,"citation_trend":[{"year":2012,"count":5},{"year":2013,"count":4},{"year":2014,"count":5},{"year":2015,"count":4},{"year":2016,"count":6},{"year":2017,"count":1},{"year":2018,"count":1},{"year":2019,"count":4},{"year":2020,"count":3},{"year":2022,"count":1},{"year":2023,"count":3},{"year":2024,"count":1},{"year":2025,"count":2}],"oa_status":"green","license":"cc-by-sa","oa_locations":[{"url":"https://doaj.org/article/76b97a46d1dc490c8747378ca5d0ac29","host_type":"repository"},{"url":"https://doaj.org/article/76b97a46d1dc490c8747378ca5d0ac29","host_type":"repository"},{"url":"https://api.elsevier.com/content/article/PII:S0969996108003215?httpAccept=text/xml","host_type":"publisher"},{"url":"https://api.elsevier.com/content/article/PII:S0969996108003215?httpAccept=text/plain","host_type":"publisher"},{"url":"https://doi.org/10.1016/j.nbd.2008.12.009","host_type":"journal"},{"url":"https://pubmed.ncbi.nlm.nih.gov/19211034","host_type":"repository"},{"url":"https://www.ncbi.nlm.nih.gov/pmc/articles/4209964","host_type":"repository"}],"fields_of_study":["Genetic Neurodegenerative Diseases","Muscle Physiology and Disorders","Neurogenetic and Muscular Disorders Research"],"mesh_terms":["Analysis of Variance","Androgens","Animals","Disease Models, Animal","Female","Male","Mice, Transgenic","Motor Activity","Motor Neurons","Receptors, Androgen","Testosterone","Gene Expression","Muscle, Skeletal","Recovery of Function","Mice","Bulbo-Spinal Atrophy, X-Linked"],"keywords":["Spinal and bulbar muscular atrophy","Androgen receptor","Denervation","Androgen","Biology","Phenotype","Internal medicine","Endocrinology","Atrophy","Trinucleotide repeat expansion","Skeletal muscle","Motor neuron","Neuroscience","Medicine","Gene","Genetics","Spinal cord","Allele"],"sdg_mappings":[{"sdg_number":0,"sdg_label":"Good health and well-being"}],"linked_datasets":[],"clinical_trials":[],"software_tools":[],"database_accessions":[],"source":"live","citation_network_status":"fetched"},"created_at":"2026-08-12T19:07:37.051974Z","pmid":null,"pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}