{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T14:18:48Z","timestamp":1791296328551,"version":"4.1.0"},"reference-count":56,"publisher":"Oxford University Press (OUP)","issue":"9","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2011,5,1]]},"DOI":"10.1093\/hmg\/ddr067","type":"journal-article","created":{"date-parts":[[2011,2,16]],"date-time":"2011-02-16T20:38:51Z","timestamp":1297888731000},"page":"1844-1853","source":"Crossref","is-referenced-by-count":96,"title":["Increased IGF-1 in muscle modulates the phenotype of severe SMA mice"],"prefix":"10.1093","volume":"20","author":[{"given":"Marta","family":"Bosch-Marc\u00e9","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claribel D.","family":"Wee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tara L.","family":"Martinez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Celeste E.","family":"Lipkes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dong W.","family":"Choe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lingling","family":"Kong","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James P.","family":"Van Meerbeke","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"Musar\u00f2","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charlotte J.","family":"Sumner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2011,2,16]]},"reference":[{"key":"key\n\t\t\t\t20170522130021_DDR067C1","volume-title":"Muscle Disorders in Childhood","author":"Dubowitz","year":"1995"},{"key":"key\n\t\t\t\t20170522130021_DDR067C2","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1006\/nbdi.1996.0010","article-title":"The neurobiology of childhood spinal muscular atrophy","volume":"3","author":"Crawford","year":"1996","journal-title":"Neurobiol. Dis."},{"key":"key\n\t\t\t\t20170522130021_DDR067C3","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1016\/0092-8674(95)90460-3","article-title":"Identification and characterization of a spinal muscular atrophy-determining gene","volume":"80","author":"Lefebvre","year":"1995","journal-title":"Cell"},{"key":"key\n\t\t\t\t20170522130021_DDR067C4","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1093\/brain\/113.2.433","article-title":"Acute infantile spinal muscular atrophy. Muscle apoptosis as a proposed pathogenetic mechanism","volume":"113","author":"Fidzianska","year":"1990","journal-title":"Brain"},{"key":"key\n\t\t\t\t20170522130021_DDR067C5","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1111\/j.1365-2990.2008.00950.x","article-title":"Childhood spinal muscular atrophy induces alterations in contractile and regulatory protein isoform expressions","volume":"34","author":"Stevens","year":"2008","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"key\n\t\t\t\t20170522130021_DDR067C6","doi-asserted-by":"crossref","first-page":"953","DOI":"10.1523\/JNEUROSCI.3237-07.2008","article-title":"Exercise-induced activation of NMDA receptor promotes motor unit development and survival in a type 2 spinal muscular atrophy model mouse","volume":"28","author":"Biondi","year":"2008","journal-title":"J. Neurosci."},{"key":"key\n\t\t\t\t20170522130021_DDR067C7","doi-asserted-by":"crossref","first-page":"842","DOI":"10.1523\/JNEUROSCI.4434-08.2009","article-title":"Impaired synaptic vesicle release and immaturity of neuromuscular junctions in spinal muscular atrophy mice","volume":"29","author":"Kong","year":"2009","journal-title":"J. Neurosci."},{"key":"key\n\t\t\t\t20170522130021_DDR067C8","doi-asserted-by":"crossref","first-page":"1063","DOI":"10.1093\/hmg\/ddm379","article-title":"Neuronal SMN expression corrects spinal muscular atrophy in severe SMA mice while muscle-specific SMN expression has no phenotypic effect","volume":"17","author":"Gavrilina","year":"2008","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522130021_DDR067C9","doi-asserted-by":"crossref","first-page":"12005","DOI":"10.1523\/JNEUROSCI.2208-10.2010","article-title":"Reduced survival of motor neuron (SMN) protein in motor neuronal progenitors functions cell autonomously to cause spinal muscular atrophy in model mice expressing the human centromeric (SMN2) gene","volume":"30","author":"Park","year":"2010","journal-title":"J. Neurosci."},{"key":"key\n\t\t\t\t20170522130021_DDR067C10","doi-asserted-by":"crossref","first-page":"571","DOI":"10.1083\/jcb.200210117","article-title":"Intact satellite cells lead to remarkable protection against Smn gene defect in differentiated skeletal muscle","volume":"161","author":"Nicole","year":"2003","journal-title":"J. Cell Biol."},{"key":"key\n\t\t\t\t20170522130021_DDR067C11","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1016\/j.yexcr.2005.08.019","article-title":"Hypomorphic Smn knockdown C2C12 myoblasts reveal intrinsic defects in myoblast fusion and myotube morphology","volume":"311","author":"Shafey","year":"2005","journal-title":"Exp. Cell Res."},{"key":"key\n\t\t\t\t20170522130021_DDR067C12","doi-asserted-by":"crossref","first-page":"694","DOI":"10.1016\/S0140-6736(95)90869-2","article-title":"Constitutive muscular abnormalities in culture in spinal muscular atrophy","volume":"345","author":"Braun","year":"1995","journal-title":"Lancet"},{"key":"key\n\t\t\t\t20170522130021_DDR067C13","doi-asserted-by":"crossref","first-page":"663","DOI":"10.1002\/(SICI)1097-4547(19980915)53:6<663::AID-JNR4>3.0.CO;2-3","article-title":"Muscle could be the therapeutic target in SMA treatment","volume":"53","author":"Guettier-Sigrist","year":"1998","journal-title":"J. Neurosci. Res."},{"key":"key\n\t\t\t\t20170522130021_DDR067C14","doi-asserted-by":"crossref","first-page":"e3209","DOI":"10.1371\/journal.pone.0003209","article-title":"Modeling spinal muscular atrophy in Drosophila","volume":"3","author":"Chang","year":"2008","journal-title":"PLoS ONE"},{"key":"key\n\t\t\t\t20170522130021_DDR067C15","doi-asserted-by":"crossref","first-page":"344","DOI":"10.1016\/j.ygcen.2010.04.009","article-title":"Insulin-like growth factors (IGFs), IGF receptors, and IGF-binding proteins: roles in skeletal muscle growth and differentiation","volume":"167","author":"Duan","year":"2010","journal-title":"Gen. Comp. Endocrinol."},{"key":"key\n\t\t\t\t20170522130021_DDR067C16","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1038\/84839","article-title":"Localized Igf-1 transgene expression sustains hypertrophy and regeneration in senescent skeletal muscle","volume":"27","author":"Musaro","year":"2001","journal-title":"Nat. Genet."},{"key":"key\n\t\t\t\t20170522130021_DDR067C17","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1083\/jcb.200108071","article-title":"Muscle-specific expression of insulin-like growth factor I counters muscle decline in mdx mice","volume":"157","author":"Barton","year":"2002","journal-title":"J. Cell Biol."},{"key":"key\n\t\t\t\t20170522130021_DDR067C18","doi-asserted-by":"crossref","first-page":"1307","DOI":"10.1083\/jcb.110.4.1307","article-title":"Nerve sprouting in innervated adult skeletal muscle induced by exposure to elevated levels of insulin-like growth factors","volume":"110","author":"Caroni","year":"1990","journal-title":"J. Cell Biol."},{"key":"key\n\t\t\t\t20170522130021_DDR067C19","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1083\/jcb.200407021","article-title":"Muscle expression of a local Igf-1 isoform protects motor neurons in an ALS mouse model","volume":"168","author":"Dobrowolny","year":"2005","journal-title":"J. Cell Biol."},{"key":"key\n\t\t\t\t20170522130021_DDR067C20","doi-asserted-by":"crossref","first-page":"839","DOI":"10.1126\/science.1086137","article-title":"Retrograde viral delivery of IGF-1 prolongs survival in a mouse ALS model","volume":"301","author":"Kaspar","year":"2003","journal-title":"Science"},{"key":"key\n\t\t\t\t20170522130021_DDR067C21","doi-asserted-by":"crossref","first-page":"316","DOI":"10.1016\/j.neuron.2009.07.019","article-title":"Overexpression of IGF-1 in muscle attenuates disease in a mouse model of spinal and bulbar muscular atrophy","volume":"63","author":"Palazzolo","year":"2009","journal-title":"Neuron"},{"key":"key\n\t\t\t\t20170522130021_DDR067C22","doi-asserted-by":"crossref","first-page":"845","DOI":"10.1093\/hmg\/ddi078","article-title":"SMNDelta7, the major product of the centromeric survival motor neuron (SMN2) gene, extends survival in mice with spinal muscular atrophy and associates with full-length SMN","volume":"14","author":"Le","year":"2005","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522130021_DDR067C23","doi-asserted-by":"crossref","first-page":"2552","DOI":"10.1093\/hmg\/ddn156","article-title":"Reduced SMN protein impairs maturation of the neuromuscular junctions in mouse models of spinal muscular atrophy","volume":"17","author":"Kariya","year":"2008","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522130021_DDR067C24","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1016\/j.expneurol.2008.02.025","article-title":"Identification of a battery of tests for drug candidate evaluation in the SMNDelta7 neonate model of spinal muscular atrophy","volume":"212","author":"El-Khodor","year":"2008","journal-title":"Exp. Neurol."},{"key":"key\n\t\t\t\t20170522130021_DDR067C25","doi-asserted-by":"crossref","first-page":"1310","DOI":"10.1093\/hmg\/ddp030","article-title":"Translational readthrough by the aminoglycoside geneticin (G418) modulates SMN stability in vitro and improves motor function in SMA mice in vivo","volume":"18","author":"Heier","year":"2009","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522130021_DDR067C26","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1172\/JCI29562","article-title":"Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophy","volume":"117","author":"Avila","year":"2007","journal-title":"J. Clin. Invest."},{"key":"key\n\t\t\t\t20170522130021_DDR067C27","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1002\/ana.21449","article-title":"Sustained improvement of spinal muscular atrophy mice treated with trichostatin a plus nutrition","volume":"64","author":"Narver","year":"2008","journal-title":"Ann. Neurol."},{"key":"key\n\t\t\t\t20170522130021_DDR067C28","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1101\/sqb.2006.71.001","article-title":"The SMN complex: an assembly machine for RNPs","volume":"71","author":"Battle","year":"2006","journal-title":"Cold Spring Harb. Symp. Quant. Biol."},{"key":"key\n\t\t\t\t20170522130021_DDR067C29","doi-asserted-by":"crossref","first-page":"340","DOI":"10.1038\/sj.embor.7400941","article-title":"Chaperoning ribonucleoprotein biogenesis in health and disease","volume":"8","author":"Pellizzoni","year":"2007","journal-title":"EMBO Rep."},{"key":"key\n\t\t\t\t20170522130021_DDR067C30","doi-asserted-by":"crossref","first-page":"e921","DOI":"10.1371\/journal.pone.0000921","article-title":"Ribonucleoprotein assembly defects correlate with spinal muscular atrophy severity and preferentially affect a subset of spliceosomal snRNPs","volume":"2","author":"Gabanella","year":"2007","journal-title":"PLoS ONE"},{"key":"key\n\t\t\t\t20170522130021_DDR067C31","doi-asserted-by":"crossref","first-page":"585","DOI":"10.1016\/j.cell.2008.03.031","article-title":"SMN deficiency causes tissue-specific perturbations in the repertoire of snRNAs and widespread defects in splicing","volume":"133","author":"Zhang","year":"2008","journal-title":"Cell"},{"key":"key\n\t\t\t\t20170522130021_DDR067C32","doi-asserted-by":"crossref","first-page":"e1000773","DOI":"10.1371\/journal.pgen.1000773","article-title":"Alternative splicing events are a late feature of pathology in a mouse model of spinal muscular atrophy","volume":"5","author":"Baumer","year":"2009","journal-title":"PLoS Genet."},{"key":"key\n\t\t\t\t20170522130021_DDR067C33","doi-asserted-by":"crossref","first-page":"740","DOI":"10.1002\/ajmg.a.32233","article-title":"Congenital heart defects in spinal muscular atrophy type I: a clinical report of two siblings and a review of the literature","volume":"146A","author":"Menke","year":"2008","journal-title":"Am. J. Med. Genet. A"},{"key":"key\n\t\t\t\t20170522130021_DDR067C34","doi-asserted-by":"crossref","first-page":"635","DOI":"10.1136\/jmg.2008.057950","article-title":"Congenital heart disease is a feature of severe infantile spinal muscular atrophy","volume":"45","author":"Rudnik-Schoneborn","year":"2008","journal-title":"J. Med. Genet."},{"key":"key\n\t\t\t\t20170522130021_DDR067C35","doi-asserted-by":"crossref","first-page":"292","DOI":"10.1016\/j.jpeds.2009.01.071","article-title":"Vascular perfusion abnormalities in infants with spinal muscular atrophy","volume":"155","author":"Araujo Ade","year":"2009","journal-title":"J. Pediatr."},{"key":"key\n\t\t\t\t20170522130021_DDR067C36","doi-asserted-by":"crossref","first-page":"3895","DOI":"10.1093\/hmg\/ddq300","article-title":"Early heart failure in the SMN{Delta}7 model of spinal muscular atrophy and correction by postnatal scAAV9-SMN delivery","volume":"19","author":"Bevan","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522130021_DDR067C37","doi-asserted-by":"crossref","first-page":"4059","DOI":"10.1093\/hmg\/ddq329","article-title":"Cardiac defects contribute to the pathology of spinal muscular atrophy models","volume":"19","author":"Shababi","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522130021_DDR067C38","doi-asserted-by":"crossref","first-page":"3906","DOI":"10.1093\/hmg\/ddq330","article-title":"Arrhythmia and cardiac defects are a feature of spinal muscular atrophy model mice","volume":"19","author":"Heier","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522130021_DDR067C39","doi-asserted-by":"crossref","first-page":"144","DOI":"10.1002\/mus.21654","article-title":"Digital necroses and vascular thrombosis in severe spinal muscular atrophy","volume":"42","author":"Rudnik-Schoneborn","year":"2010","journal-title":"Muscle Nerve"},{"key":"key\n\t\t\t\t20170522130021_DDR067C40","doi-asserted-by":"crossref","first-page":"791","DOI":"10.1139\/h06-054","article-title":"The ABCs of IGF-I isoforms: impact on muscle hypertrophy and implications for repair","volume":"31","author":"Barton","year":"2006","journal-title":"Appl. Physiol. Nutr. Metab."},{"key":"key\n\t\t\t\t20170522130021_DDR067C41","doi-asserted-by":"crossref","first-page":"960","DOI":"10.1242\/jcs.061119","article-title":"A growth stimulus is needed for IGF-1 to induce skeletal muscle hypertrophy in vivo","volume":"123","author":"Shavlakadze","year":"2010","journal-title":"J. Cell Sci."},{"key":"key\n\t\t\t\t20170522130021_DDR067C42","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1016\/S0140-6736(95)91439-0","article-title":"Hypoglycaemia in spinal muscular atrophy","volume":"346","author":"Bruce","year":"1995","journal-title":"Lancet"},{"key":"key\n\t\t\t\t20170522130021_DDR067C43","doi-asserted-by":"crossref","first-page":"997","DOI":"10.1212\/01.WNL.0000086813.59722.72","article-title":"Patients with severe muscle wasting are prone to develop hypoglycemia during fasting","volume":"61","author":"Orngreen","year":"2003","journal-title":"Neurology"},{"key":"key\n\t\t\t\t20170522130021_DDR067C44","doi-asserted-by":"crossref","first-page":"835","DOI":"10.1016\/j.bbrc.2009.11.148","article-title":"Effect of diet on the survival and phenotype of a mouse model for spinal muscular atrophy","volume":"391","author":"Butchbach","year":"2010","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"key\n\t\t\t\t20170522130021_DDR067C45","doi-asserted-by":"crossref","first-page":"1931","DOI":"10.1212\/01.wnl.0000290830.40544.b9","article-title":"The changing natural history of spinal muscular atrophy type 1","volume":"69","author":"Oskoui","year":"2007","journal-title":"Neurology"},{"key":"key\n\t\t\t\t20170522130021_DDR067C46","doi-asserted-by":"crossref","first-page":"3145","DOI":"10.1093\/hmg\/ddp253","article-title":"Inhibition of myostatin does not ameliorate disease features of severe spinal muscular atrophy mice","volume":"18","author":"Sumner","year":"2009","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522130021_DDR067C47","doi-asserted-by":"crossref","first-page":"11288","DOI":"10.1523\/JNEUROSCI.1764-10.2010","article-title":"In vivo NMDA receptor activation accelerates motor unit maturation, protects spinal motor neurons, and enhances SMN2 gene expression in severe spinal muscular atrophy mice","volume":"30","author":"Biondi","year":"2010","journal-title":"J. Neurosci."},{"key":"key\n\t\t\t\t20170522130021_DDR067C48","doi-asserted-by":"crossref","first-page":"e15457","DOI":"10.1371\/journal.pone.0015457","article-title":"Synaptic defects in the spinal and neuromuscular circuitry in a mouse model of spinal muscular atrophy","volume":"5","author":"Ling","year":"2010","journal-title":"PLoS ONE"},{"key":"key\n\t\t\t\t20170522130021_DDR067C49","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1016\/j.neuron.2010.12.032","article-title":"Early functional impairment of sensory-motor connectivity in a mouse model of spinal muscular atrophy","volume":"69","author":"Mentis","year":"2010","journal-title":"Neuron"},{"key":"key\n\t\t\t\t20170522130021_DDR067C50","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1016\/j.exger.2006.05.004","article-title":"The neuroprotective effects of a locally acting IGF-1 isoform","volume":"42","author":"Musaro","year":"2007","journal-title":"Exp. Gerontol."},{"key":"key\n\t\t\t\t20170522130021_DDR067C51","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1038\/276401a0","article-title":"Change in synaptic channel gating during neuromuscular development","volume":"276","author":"Sakmann","year":"1978","journal-title":"Nature"},{"key":"key\n\t\t\t\t20170522130021_DDR067C52","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1113\/jphysiol.1980.sp013275","article-title":"A post-natal decrease in acetylcholine channel open time at rat end-plates","volume":"303","author":"Fischbach","year":"1980","journal-title":"J. Physiol."},{"key":"key\n\t\t\t\t20170522130021_DDR067C53","doi-asserted-by":"crossref","first-page":"1351","DOI":"10.1523\/JNEUROSCI.23-04-01351.2003","article-title":"Target-derived trophic effect on skeletal muscle innervation in senescent mice","volume":"23","author":"Messi","year":"2003","journal-title":"J. Neurosci."},{"key":"key\n\t\t\t\t20170522130021_DDR067C54","doi-asserted-by":"crossref","first-page":"973","DOI":"10.1093\/hmg\/ddp562","article-title":"Ciliary neurotrophic factor-induced sprouting preserves motor function in a mouse model of mild spinal muscular atrophy","volume":"19","author":"Simon","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522130021_DDR067C55","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1083\/jcb.200208079","article-title":"A transgene carrying an A2G missense mutation in the SMN gene modulates phenotypic severity in mice with severe (type I) spinal muscular atrophy","volume":"160","author":"Monani","year":"2003","journal-title":"J. Cell Biol."},{"key":"key\n\t\t\t\t20170522130021_DDR067C56","doi-asserted-by":"crossref","first-page":"1171","DOI":"10.1093\/hmg\/ddi130","article-title":"The role of histone acetylation in SMN gene expression","volume":"14","author":"Kernochan","year":"2005","journal-title":"Hum. Mol. Genet."}],"container-title":["Human Molecular Genetics"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/hmg\/article-pdf\/20\/9\/1844\/17255511\/ddr067.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,6,8]],"date-time":"2019-06-08T12:00:26Z","timestamp":1559995226000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/hmg\/article-lookup\/doi\/10.1093\/hmg\/ddr067"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,2,16]]},"references-count":56,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2011,2,16]]},"published-print":{"date-parts":[[2011,5,1]]}},"URL":"https:\/\/doi.org\/10.1093\/hmg\/ddr067","relation":{"has-review":[{"id-type":"doi","id":"10.3410\/f.10854956.11772054","asserted-by":"object"}]},"ISSN":["1460-2083","0964-6906"],"issn-type":[{"value":"1460-2083","type":"electronic"},{"value":"0964-6906","type":"print"}],"subject":[],"published":{"date-parts":[[2011,2,16]]}}}