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It is as yet unclear whether a decreased capacity of <jats:styled-content style=\"fixed-case\">SMN<\/jats:styled-content> in sn<jats:styled-content style=\"fixed-case\">RNP<\/jats:styled-content> assembly, and, hence, transcriptome abnormalities, account for the specific neuromuscular phenotype in <jats:styled-content style=\"fixed-case\">SMA<\/jats:styled-content>. Across metazoa, the <jats:styled-content style=\"fixed-case\">SMN<\/jats:styled-content>\u2010Gemins complex concentrates in multiple nuclear gems that frequently neighbour or overlap Cajal bodies. The number of gems has long been known to be a faithful indicator of <jats:styled-content style=\"fixed-case\">SMN<\/jats:styled-content> levels, which are linked to <jats:styled-content style=\"fixed-case\">SMA<\/jats:styled-content> severity. Intriguingly, a flurry of recent studies have revealed that depletion of this nuclear structure is also a signature feature of amyotrophic lateral sclerosis (<jats:styled-content style=\"fixed-case\">ALS<\/jats:styled-content>), the most common adult\u2010onset motor neuron disease. This review discusses such a surprising crossover in addition to highlighting the most recent work on the intricate world of spliceosome building, which seems to be at the heart of motor neuron physiology and survival.<\/jats:p>","DOI":"10.1111\/cns.12242","type":"journal-article","created":{"date-parts":[[2014,3,19]],"date-time":"2014-03-19T14:19:50Z","timestamp":1395238790000},"page":"574-581","source":"Crossref","is-referenced-by-count":28,"title":["Gem Depletion: Amyotrophic Lateral Sclerosis and Spinal Muscular Atrophy Crossover"],"prefix":"10.1111","volume":"20","author":[{"given":"Ruben J.","family":"Cauchi","sequence":"first","affiliation":[{"name":"Department of Physiology and Biochemistry University of Malta Msida 2080 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