{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T06:51:10Z","timestamp":1791960670410,"version":"4.3.4"},"reference-count":328,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2021,2,9]],"date-time":"2021-02-09T00:00:00Z","timestamp":1612828800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["NS101958"],"award-info":[{"award-number":["NS101958"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Substantial evidence indicates that mitochondrial impairment contributes to neuronal dysfunction and vulnerability in disease states, leading investigators to propose that the enhancement of mitochondrial function should be considered a strategy for neuroprotection. However, multiple attempts to improve mitochondrial function have failed to impact disease progression, suggesting that the biology underlying the normal regulation of mitochondrial pathways in neurons, and its dysfunction in disease, is more complex than initially thought. Here, we present the proteins and associated pathways involved in the transcriptional regulation of nuclear-encoded genes for mitochondrial function, with a focus on the transcriptional coactivator peroxisome proliferator-activated receptor gamma coactivator-1alpha (PGC-1\u03b1). We highlight PGC-1\u03b1\u2019s roles in neuronal and non-neuronal cell types and discuss evidence for the dysregulation of PGC-1\u03b1-dependent pathways in Huntington\u2019s Disease, Parkinson\u2019s Disease, and developmental disorders, emphasizing the relationship between disease-specific cellular vulnerability and cell-type-specific patterns of PGC-1\u03b1 expression. Finally, we discuss the challenges inherent to therapeutic targeting of PGC-1\u03b1-related transcriptional programs, considering the roles for neuron-enriched transcriptional coactivators in co-regulating mitochondrial and synaptic genes. This information will provide novel insights into the unique aspects of transcriptional regulation of mitochondrial function in neurons and the opportunities for therapeutic targeting of transcriptional pathways for neuroprotection.<\/jats:p>","DOI":"10.3390\/cells10020352","type":"journal-article","created":{"date-parts":[[2021,2,9]],"date-time":"2021-02-09T23:43:16Z","timestamp":1612914196000},"page":"352","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":50,"title":["Dysregulation of PGC-1\u03b1-Dependent Transcriptional Programs in Neurological and Developmental Disorders: Therapeutic Challenges and Opportunities"],"prefix":"10.3390","volume":"10","author":[{"given":"Laura J.","family":"McMeekin","sequence":"first","affiliation":[{"name":"Department of Neuroscience, Life Sciences Division, Southern Research, Birmingham, AL 35205, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2170-8342","authenticated-orcid":false,"given":"Stephanie N.","family":"Fox","sequence":"additional","affiliation":[{"name":"Department of Cell, Developmental, and Integrative Biology, University of Alabama at Birmingham, Birmingham, AL 35294, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephanie M.","family":"Boas","sequence":"additional","affiliation":[{"name":"Department of Cell, Developmental, and Integrative Biology, University of Alabama at Birmingham, Birmingham, AL 35294, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rita M.","family":"Cowell","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Life Sciences Division, Southern Research, Birmingham, AL 35205, USA"},{"name":"Department of Cell, Developmental, and Integrative Biology, University of Alabama at Birmingham, Birmingham, AL 35294, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,2,9]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1113\/eph8802500","article-title":"Regulation and co-ordination of nuclear gene expression during mitochondrial biogenesis","volume":"88","author":"Goffart","year":"2003","journal-title":"Exp. 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