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While mutations in a large number of molecules associated with lipid metabolism are known to be implicated in MNDs, there remains a lack of clarity regarding the key functional pathways involved, and their inter-relationships. This review highlights evidence that defines defects within two specific lipid (cholesterol\/oxysterol and phosphatidylethanolamine) biosynthetic cascades as being centrally involved in MND, particularly hereditary spastic paraplegia. We also identify how other MND-associated molecules may impact these cascades, in particular through impaired organellar interfacing, to propose \u2018subcellular lipidome imbalance\u2019 as a likely common pathomolecular theme in MND. Further exploration of this mechanism has the potential to identify new therapeutic targets and management strategies for modulation of disease progression in hereditary spastic paraplegias and other MNDs.<\/jats:p>","DOI":"10.1093\/brain\/awz382","type":"journal-article","created":{"date-parts":[[2019,11,13]],"date-time":"2019-11-13T12:10:04Z","timestamp":1573647004000},"page":"1073-1087","source":"Crossref","is-referenced-by-count":57,"title":["Lipid metabolic pathways converge in motor neuron degenerative diseases"],"prefix":"10.1093","volume":"143","author":[{"given":"Olivia J","family":"Rickman","sequence":"first","affiliation":[{"name":"Medical Research (Level 4), RILD Wellcome Wolfson Centre, University of Exeter Medical School, Royal Devon and Exeter NHS Foundation Trust, Barrack Road, Exeter, EX2 5DW, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emma L","family":"Baple","sequence":"first","affiliation":[{"name":"Medical Research (Level 4), RILD Wellcome Wolfson Centre, University of Exeter Medical School, Royal Devon and Exeter NHS Foundation Trust, Barrack Road, Exeter, EX2 5DW, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew H","family":"Crosby","sequence":"first","affiliation":[{"name":"Medical Research (Level 4), RILD Wellcome Wolfson Centre, University of Exeter Medical School, Royal Devon and Exeter NHS Foundation Trust, Barrack Road, Exeter, EX2 5DW, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2019,12,18]]},"reference":[{"key":"2020042119474855400_awz382-B1","doi-asserted-by":"crossref","first-page":"525","DOI":"10.1083\/jcb.201602007","article-title":"MICOS and phospholipid transfer by Ups2-Mdm35 organize membrane lipid synthesis in mitochondria","volume":"213","author":"Aaltonen","year":"2016","journal-title":"J Cell Biol"},{"key":"2020042119474855400_awz382-B2","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1016\/j.biochi.2018.06.020","article-title":"Identification of 7\u03b1,24-dihydroxy-3-oxocholest-4-en-26-oic and 7\u03b1,25-dihydroxy-3-oxocholest-4-en-26-oic acids in human cerebrospinal fluid and plasma","volume":"153","author":"Abdel-Khalik","year":"2018","journal-title":"Biochimie"},{"key":"2020042119474855400_awz382-B3","first-page":"547","article-title":"A mutation of EPT1 (SELENOI) underlies a new disorder of Kennedy pathway phospholipid biosynthesis","volume":"140","author":"Ahmed","year":"2017","journal-title":"Brain"},{"key":"2020042119474855400_awz382-B4","doi-asserted-by":"crossref","first-page":"679","DOI":"10.1016\/j.bbadis.2014.08.010","article-title":"Sporadic and hereditary amyotrophic lateral sclerosis (ALS)","volume":"1852","author":"Ajroud-Driss","year":"2015","journal-title":"Biochim Biophys Acta"},{"key":"2020042119474855400_awz382-B5","doi-asserted-by":"crossref","first-page":"913","DOI":"10.1002\/ana.22534","article-title":"A mutation in sigma-1 receptor causes juvenile amyotrophic lateral sclerosis","volume":"70","author":"Al-Saif","year":"2011","journal-title":"Ann Neurol"},{"key":"2020042119474855400_awz382-B6","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1007\/s10048-011-0291-8","article-title":"A nullimorphic ERLIN2 mutation defines a complicated hereditary spastic paraplegia locus (SPG18)","volume":"12","author":"Alazami","year":"2011","journal-title":"Neurogenetics"},{"key":"2020042119474855400_awz382-B7","doi-asserted-by":"crossref","first-page":"1033","DOI":"10.1194\/jlr.M034124","article-title":"On the regulatory role of side-chain hydroxylated oxysterols in the brain. 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