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This review provides an overview of the work that led to the discovery of neurosteroids, our current understanding of their intracellular biosynthetic machinery, and their roles in regulating the development and function of nervous tissue. Neurosteroids mediate signalling in the brain via multiple mechanisms. Here, we describe in detail their effects on GABA (inhibitory) and NMDA (excitatory) receptors, two signalling pathways of opposing function. Furthermore, emerging evidence points to altered neurosteroid function and signalling in neurological disease. This review focuses on neurodegenerative diseases associated with altered neurosteroid metabolism, mainly Niemann-Pick type C, multiple sclerosis and Alzheimer disease. Finally, we summarise the use of natural and synthetic neurosteroids as current and emerging therapeutics alongside their potential use as disease biomarkers.<\/jats:p>","DOI":"10.1042\/ebc20200043","type":"journal-article","created":{"date-parts":[[2020,8,5]],"date-time":"2020-08-05T09:23:59Z","timestamp":1596619439000},"page":"591-606","update-policy":"https:\/\/doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":67,"title":["Biosynthesis and signalling functions of central and peripheral nervous system neurosteroids in health and disease"],"prefix":"10.1042","volume":"64","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3626-1611","authenticated-orcid":false,"given":"Emyr","family":"Lloyd-Evans","sequence":"additional","affiliation":[{"name":"Sir Martin Evans Building, School of Biosciences, Cardiff University, Museum Avenue, Cardiff, CF10 3AX, U.K."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4133-6064","authenticated-orcid":false,"given":"Helen","family":"Waller-Evans","sequence":"additional","affiliation":[{"name":"Medicines Discovery Institute, Main Building, Cardiff University, Park Place, Cardiff, CF10 3AT, U.K."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"288","published-online":{"date-parts":[[2020,8,5]]},"reference":[{"key":"2021111610305678700_B1","doi-asserted-by":"publisher","first-page":"113051","DOI":"10.1016\/j.ygcen.2018.10.014","article-title":"Kobayashi award: Discovery of cerebellar and pineal neurosteroids and their biological actions on the growth and survival of Purkinje cells during development (review)","volume":"284","author":"Tsutsui","year":"2019","journal-title":"Gen. 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