{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T21:02:20Z","timestamp":1790370140855,"version":"4.1.0"},"reference-count":34,"publisher":"Portland Press Ltd.","issue":"6","content-domain":{"domain":["portlandpress.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2009,12,1]]},"abstract":"<jats:p>Translation elongation factor eEF1A (eukaryotic elongation factor 1A) exists as two individually encoded variants in mammals, which are 98% similar and 92% identical at the amino acid level. One variant, eEF1A1, is almost ubiquitously expressed, the other variant, eEF1A2, shows a very restricted pattern of expression. A spontaneous mutation was described in 1972, which gives rise to the wasted phenotype: homozygous wst\/wst mice develop normally until shortly after weaning, but then lose muscle bulk, acquire tremors and gait abnormalities and die by 4\u00a0weeks. This mutation has been shown to be a deletion of 15\u00a0kb that removes the promoter and first exon of the gene encoding eEF1A2. The reciprocal pattern of expression of eEF1A1 and eEF1A2 in muscle fits well with the timing of onset of the phenotype of wasted mice: eEF1A1 declines after birth until it is undetectable by 3\u00a0weeks, whereas eEF1A2 expression increases over this time. No other gene is present in the wasted deletion, and transgenic studies have shown that the phenotype is due to loss of eEF1A2. We have shown that eEF1A2, but not eEF1A1, is also expressed at high levels in motor neurons in the spinal cord. Wasted mice develop many pathological features of motor neuron degeneration and may represent a good model for early onset of motor neuron disease. Molecular modelling of the eEF1A1 and eEF1A2 protein structures highlights differences between the two variants that may be critical for functional differences. Interactions between eEF1A2 and ZPR1 (zinc-finger protein 1), which interacts with the SMN (survival motor neuron) protein, may be important in motor neuron biology.<\/jats:p>","DOI":"10.1042\/bst0371293","type":"journal-article","created":{"date-parts":[[2009,11,19]],"date-time":"2009-11-19T18:02:29Z","timestamp":1258653749000},"page":"1293-1297","update-policy":"https:\/\/doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":33,"title":["eEF1A2 and neuronal degeneration"],"prefix":"10.1042","volume":"37","author":[{"given":"Catherine\u00a0M.","family":"Abbott","sequence":"first","affiliation":[{"name":"Medical Genetics Section, Molecular Medicine Centre, Institute of Genetics and Molecular Medicine, Western General Hospital, University of Edinburgh, Edinburgh EH4 2XU, U.K."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helen\u00a0J.","family":"Newbery","sequence":"additional","affiliation":[{"name":"Medical Genetics Section, Molecular Medicine Centre, Institute of Genetics and Molecular Medicine, Western General Hospital, University of Edinburgh, Edinburgh EH4 2XU, U.K."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charlotte\u00a0E.","family":"Squires","sequence":"additional","affiliation":[{"name":"Medical Genetics Section, Molecular Medicine Centre, Institute of Genetics and Molecular Medicine, Western General Hospital, University of Edinburgh, Edinburgh EH4 2XU, U.K."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Brownstein","sequence":"additional","affiliation":[{"name":"Research Animal Pathology Core Facility, Room W3.03, Queen's Medical Research Institute, 47 Little France Crescent, University of Edinburgh, Edinburgh EH16 4TJ, U.K."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lowri\u00a0A.","family":"Griffiths","sequence":"additional","affiliation":[{"name":"Medical Genetics Section, Molecular Medicine Centre, Institute of Genetics and Molecular Medicine, Western General Hospital, University of Edinburgh, Edinburgh EH4 2XU, U.K."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dinesh\u00a0C.","family":"Soares","sequence":"additional","affiliation":[{"name":"Medical Genetics Section, Molecular Medicine Centre, Institute of Genetics and Molecular Medicine, Western General Hospital, University of Edinburgh, Edinburgh EH4 2XU, U.K."},{"name":"School of Chemistry, University of Edinburgh, Joseph Black Building, King's Buildings, West Mains Road, Edinburgh EH9 3JJ, U.K."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"288","published-online":{"date-parts":[[2009,11,19]]},"reference":[{"key":"2021111719334195100_B1","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1016\/j.tibs.2003.11.006","article-title":"Translation factors: in sickness and in health","volume":"29","author":"Abbott","year":"2004","journal-title":"Trends Biochem. 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