{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T19:43:44Z","timestamp":1792784624095,"version":"4.5.0"},"reference-count":69,"publisher":"Elsevier BV","issue":"2","license":[{"start":{"date-parts":[[2011,8,1]],"date-time":"2011-08-01T00:00:00Z","timestamp":1312156800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2011,8,1]],"date-time":"2011-08-01T00:00:00Z","timestamp":1312156800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2013,8,22]],"date-time":"2013-08-22T00:00:00Z","timestamp":1377129600000},"content-version":"vor","delay-in-days":752,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"},{"start":{"date-parts":[[2011,8,1]],"date-time":"2011-08-01T00:00:00Z","timestamp":1312156800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2011,8,1]],"date-time":"2011-08-01T00:00:00Z","timestamp":1312156800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2011,8,1]],"date-time":"2011-08-01T00:00:00Z","timestamp":1312156800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2011,8,1]],"date-time":"2011-08-01T00:00:00Z","timestamp":1312156800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2011,8,1]],"date-time":"2011-08-01T00:00:00Z","timestamp":1312156800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-004"}],"funder":[{"DOI":"10.13039\/100000894","name":"SMA Foundation","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100000894","id-type":"DOI","asserted-by":"crossref"}]},{"name":"NIH","award":["NS20480"],"award-info":[{"award-number":["NS20480"]}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Developmental Biology"],"published-print":{"date-parts":[[2011,8]]},"DOI":"10.1016\/j.ydbio.2011.05.667","type":"journal-article","created":{"date-parts":[[2011,6,5]],"date-time":"2011-06-05T14:41:18Z","timestamp":1307284878000},"page":"432-444","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":119,"title":["Muscles in a mouse model of spinal muscular atrophy show profound defects in neuromuscular development even in the absence of failure in neuromuscular transmission or loss of motor neurons"],"prefix":"10.1016","volume":"356","author":[{"given":"Young il","family":"Lee","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michelle","family":"Mikesh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ian","family":"Smith","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mendell","family":"Rimer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wesley","family":"Thompson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.ydbio.2011.05.667_bb0005","doi-asserted-by":"crossref","first-page":"1385","DOI":"10.1083\/jcb.150.6.1385","article-title":"Absence of alpha-syntrophin leads to structurally aberrant neuromuscular synapses deficient in utrophin","volume":"150","author":"Adams","year":"2000","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0010","doi-asserted-by":"crossref","first-page":"3433","DOI":"10.1523\/JNEUROSCI.13-08-03433.1993","article-title":"Degradation of two AChR populations at rat neuromuscular junctions: regulation in vivo by electrical stimulation","volume":"13","author":"Andreose","year":"1993","journal-title":"J. Neurosci."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0015","doi-asserted-by":"crossref","first-page":"1726","DOI":"10.1172\/JCI22922","article-title":"Lentivector-mediated SMN replacement in a mouse model of spinal muscular atrophy","volume":"114","author":"Azzouz","year":"2004","journal-title":"J. Clin. Invest."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0020","doi-asserted-by":"crossref","first-page":"587","DOI":"10.1016\/0022-3468(74)90093-1","article-title":"An experimental study of acute neonatal enterocolitis\u2014the importance of breast milk","volume":"9","author":"Barlow","year":"1974","journal-title":"J. Pediatr. Surg."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0025","doi-asserted-by":"crossref","first-page":"1453","DOI":"10.1083\/jcb.153.7.1453","article-title":"Muscle activity and muscle agrin regulate the organization of cytoskeletal proteins and attached acetylcholine receptor (AchR) aggregates in skeletal muscle fibers","volume":"153","author":"Bezakova","year":"2001","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0030","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":"10.1016\/j.ydbio.2011.05.667_bb0035","doi-asserted-by":"crossref","first-page":"367","DOI":"10.1113\/jphysiol.1987.sp016619","article-title":"Control of end-plate channel properties by neurotrophic effects and by muscle activity in rat","volume":"388","author":"Brenner","year":"1987","journal-title":"J. Physiol."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0040","doi-asserted-by":"crossref","first-page":"597","DOI":"10.1038\/nrn2670","article-title":"Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick?","volume":"10","author":"Burghes","year":"2009","journal-title":"Nat. Rev. Neurosci."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0045","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1016\/j.nbd.2007.04.009","article-title":"Abnormal motor phenotype in the SMNDelta7 mouse model of spinal muscular atrophy","volume":"27","author":"Butchbach","year":"2007","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0050","doi-asserted-by":"crossref","first-page":"421","DOI":"10.1016\/j.cell.2004.12.020","article-title":"Thrombospondins are astrocyte-secreted proteins that promote CNS synaptogenesis","volume":"120","author":"Christopherson","year":"2005","journal-title":"Cell"},{"key":"10.1016\/j.ydbio.2011.05.667_bb0055","doi-asserted-by":"crossref","first-page":"1107","DOI":"10.1083\/jcb.152.5.1107","article-title":"Deletion of murine SMN exon 7 directed to skeletal muscle leads to severe muscular dystrophy","volume":"152","author":"Cifuentes-Diaz","year":"2001","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0060","doi-asserted-by":"crossref","first-page":"1439","DOI":"10.1093\/hmg\/11.12.1439","article-title":"Neurofilament accumulation at the motor endplate and lack of axonal sprouting in a spinal muscular atrophy mouse model","volume":"11","author":"Cifuentes-Diaz","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0065","doi-asserted-by":"crossref","first-page":"237","DOI":"10.1006\/mcne.1997.0623","article-title":"Agrin-induced postsynaptic-like apparatus in skeletal muscle fibers in vivo","volume":"9","author":"Cohen","year":"1997","journal-title":"Mol. Cell. Neurosci."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0070","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1016\/0012-1606(90)90196-P","article-title":"Development of muscle fiber types in the prenatal rat hindlimb","volume":"138","author":"Condon","year":"1990","journal-title":"Dev. Biol."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0075","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1016\/0012-1606(90)90197-Q","article-title":"Differentiation of fiber types in aneural musculature of the prenatal rat hindlimb","volume":"138","author":"Condon","year":"1990","journal-title":"Dev. Biol."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0080","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":"10.1016\/j.ydbio.2011.05.667_bb0085","doi-asserted-by":"crossref","first-page":"2040","DOI":"10.1073\/pnas.90.5.2040","article-title":"Electrical activity-dependent regulation of the acetylcholine receptor delta-subunit gene, MyoD, and myogenin in primary myotubes","volume":"90","author":"Dutton","year":"1993","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0090","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1111\/j.1365-2818.2005.01466.x","article-title":"Reconstruct: a free editor for serial section microscopy","volume":"218","author":"Fiala","year":"2005","journal-title":"J. Microsc."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0095","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1038\/nbt.1610","article-title":"Rescue of the spinal muscular atrophy phenotype in a mouse model by early postnatal delivery of SMN","volume":"28","author":"Foust","year":"2010","journal-title":"Nat. Biotechnol."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0100","doi-asserted-by":"crossref","first-page":"849","DOI":"10.1093\/hmg\/9.5.849","article-title":"Nuclear targeting defect of SMN lacking the C-terminus in a mouse model of spinal muscular atrophy","volume":"9","author":"Frugier","year":"2000","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0105","doi-asserted-by":"crossref","first-page":"3629","DOI":"10.1093\/hmg\/ddi390","article-title":"The activity of the spinal muscular atrophy protein is regulated during development and cellular differentiation","volume":"14","author":"Gabanella","year":"2005","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0110","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":"10.1016\/j.ydbio.2011.05.667_bb0115","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1113\/jphysiol.1984.sp015466","article-title":"Muscle size and motor unit survival in mice","volume":"356","author":"Habgood","year":"1984","journal-title":"J. Physiol."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0120","doi-asserted-by":"crossref","first-page":"657","DOI":"10.1097\/00001756-200505120-00001","article-title":"Spinal muscular atrophy: a delayed development hypothesis","volume":"16","author":"Hausmanowa-Petrusewicz","year":"2005","journal-title":"Neuroreport"},{"key":"10.1016\/j.ydbio.2011.05.667_bb0125","doi-asserted-by":"crossref","first-page":"6873","DOI":"10.1523\/JNEUROSCI.1086-06.2006","article-title":"Induction of neuregulin signaling in mouse Schwann cells in vivo mimics responses to denervation","volume":"26","author":"Hayworth","year":"2006","journal-title":"J. Neurosci."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0130","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":"10.1016\/j.ydbio.2011.05.667_bb0135","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1038\/71709","article-title":"A mouse model for spinal muscular atrophy","volume":"24","author":"Hsieh-Li","year":"2000","journal-title":"Nat. Genet."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0140","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1093\/hmg\/9.3.341","article-title":"Reduced survival motor neuron (Smn) gene dose in mice leads to motor neuron degeneration: an animal model for spinal muscular atrophy type III","volume":"9","author":"Jablonka","year":"2000","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0145","doi-asserted-by":"crossref","first-page":"975","DOI":"10.1023\/B:NEUR.0000020636.27222.2d","article-title":"Terminal Schwann cells guide the reinnervation of muscle after nerve injury","volume":"32","author":"Kang","year":"2003","journal-title":"J. Neurocytol."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0150","doi-asserted-by":"crossref","first-page":"5948","DOI":"10.1523\/JNEUROSCI.0621-07.2007","article-title":"Regulation of the intermediate filament protein nestin at rodent neuromuscular junctions by innervation and activity","volume":"27","author":"Kang","year":"2007","journal-title":"J. Neurosci."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0155","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":"10.1016\/j.ydbio.2011.05.667_bb0160","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":"10.1016\/j.ydbio.2011.05.667_bb0165","doi-asserted-by":"crossref","first-page":"1077","DOI":"10.1083\/jcb.200401115","article-title":"Nerve-independent formation of a topologically complex postsynaptic apparatus","volume":"164","author":"Kummer","year":"2004","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0170","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":"10.1016\/j.ydbio.2011.05.667_bb0175","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":"10.1016\/j.ydbio.2011.05.667_bb0180","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":"10.1016\/j.ydbio.2011.05.667_bb0185","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1002\/dneu.20840","article-title":"Smn deficiency causes neuritogenesis and neurogenesis defects in the retinal neurons of a mouse model of spinal muscular atrophy","volume":"71","author":"Liu","year":"2011","journal-title":"Dev. Neurobiol."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0190","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1093\/hmg\/9.2.259","article-title":"An exonic enhancer is required for inclusion of an essential exon in the SMA-determining gene SMN","volume":"9","author":"Lorson","year":"2000","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0195","doi-asserted-by":"crossref","first-page":"6307","DOI":"10.1073\/pnas.96.11.6307","article-title":"A single nucleotide in the SMN gene regulates splicing and is responsible for spinal muscular atrophy","volume":"96","author":"Lorson","year":"1999","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0200","doi-asserted-by":"crossref","first-page":"9376","DOI":"10.1523\/JNEUROSCI.18-22-09376.1998","article-title":"Schwann cells proliferate at rat neuromuscular junctions during development and regeneration","volume":"18","author":"Love","year":"1998","journal-title":"J. Neurosci."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0205","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1002\/(SICI)1097-0185(199707)248:3<355::AID-AR8>3.0.CO;2-O","article-title":"Electron microscopic study of long-term denervated rat skeletal muscle","volume":"248","author":"Lu","year":"1997","journal-title":"Anat. Rec."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0210","doi-asserted-by":"crossref","first-page":"3663","DOI":"10.1523\/JNEUROSCI.20-10-03663.2000","article-title":"From plaque to pretzel: fold formation and acetylcholine receptor loss at the developing neuromuscular junction","volume":"20","author":"Marques","year":"2000","journal-title":"J. Neurosci."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0215","doi-asserted-by":"crossref","first-page":"474","DOI":"10.1097\/NEN.0b013e3181a10ea1","article-title":"The developmental pattern of myotubes in spinal muscular atrophy indicates prenatal delay of muscle maturation","volume":"68","author":"Martinez-Hernandez","year":"2009","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0220","doi-asserted-by":"crossref","first-page":"2900","DOI":"10.1093\/hmg\/ddn189","article-title":"Embryonic motor axon development in the severe SMA mouse","volume":"17","author":"McGovern","year":"2008","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0225","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":"2011","journal-title":"Neuron"},{"key":"10.1016\/j.ydbio.2011.05.667_bb0230","doi-asserted-by":"crossref","first-page":"635","DOI":"10.1016\/S0896-6273(02)01020-6","article-title":"Roles of neurotransmitter in synapse formation: development of neuromuscular junctions lacking choline acetyltransferase","volume":"36","author":"Misgeld","year":"2002","journal-title":"Neuron"},{"key":"10.1016\/j.ydbio.2011.05.667_bb0235","doi-asserted-by":"crossref","first-page":"885","DOI":"10.1016\/j.neuron.2005.12.001","article-title":"Spinal muscular atrophy: a deficiency in a ubiquitous protein; a motor neuron-specific disease","volume":"48","author":"Monani","year":"2005","journal-title":"Neuron"},{"key":"10.1016\/j.ydbio.2011.05.667_bb0240","doi-asserted-by":"crossref","first-page":"949","DOI":"10.1093\/hmg\/ddm367","article-title":"Selective vulnerability of motor neurons and dissociation of pre- and post-synaptic pathology at the neuromuscular junction in mouse models of spinal muscular atrophy","volume":"17","author":"Murray","year":"2008","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0245","doi-asserted-by":"crossref","first-page":"580","DOI":"10.1038\/nature03112","article-title":"A synaptic laminin\u2013calcium channel interaction organizes active zones in motor nerve terminals","volume":"432","author":"Nishimune","year":"2004","journal-title":"Nature"},{"key":"10.1016\/j.ydbio.2011.05.667_bb0250","doi-asserted-by":"crossref","first-page":"258","DOI":"10.1038\/374258a0","article-title":"Aberrant differentiation of neuromuscular junctions in mice lacking s-laminin\/laminin beta 2","volume":"374","author":"Noakes","year":"1995","journal-title":"Nature"},{"key":"10.1016\/j.ydbio.2011.05.667_bb0255","doi-asserted-by":"crossref","first-page":"108","DOI":"10.1007\/s11910-010-0095-5","article-title":"Spinal muscular atrophy: new and emerging insights from model mice","volume":"10","author":"Park","year":"2010","journal-title":"Curr. Neurol. Neurosci. Rep."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0260","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":"10.1016\/j.ydbio.2011.05.667_bb0265","doi-asserted-by":"crossref","first-page":"1712","DOI":"10.1086\/302160","article-title":"Intragenic telSMN mutations: frequency, distribution, evidence of a founder effect, and modification of the spinal muscular atrophy phenotype by cenSMN copy number","volume":"63","author":"Parsons","year":"1998","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0270","doi-asserted-by":"crossref","first-page":"563","DOI":"10.1016\/S0896-6273(03)00682-2","article-title":"Glial cells maintain synaptic structure and function and promote development of the neuromuscular junction in vivo","volume":"40","author":"Reddy","year":"2003","journal-title":"Neuron"},{"key":"10.1016\/j.ydbio.2011.05.667_bb0275","doi-asserted-by":"crossref","first-page":"254","DOI":"10.1006\/mcne.1997.0622","article-title":"gamma-AChR\/epsilon-AChR switch at agrin-induced postsynaptic-like apparatus in skeletal muscle","volume":"9","author":"Rimer","year":"1997","journal-title":"Mol. Cell. Neurosci."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0280","doi-asserted-by":"crossref","first-page":"3513","DOI":"10.1073\/pnas.0813112106","article-title":"Rescuing Z+agrin splicing in Nova null mice restores synapse formation and unmasks a physiologic defect in motor neuron firing","volume":"106","author":"Ruggiu","year":"2009","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0285","doi-asserted-by":"crossref","first-page":"849","DOI":"10.1523\/JNEUROSCI.4496-09.2010","article-title":"Altered intracellular Ca2+ homeostasis in nerve terminals of severe spinal muscular atrophy mice","volume":"30","author":"Ruiz","year":"2010","journal-title":"J. Neurosci."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0290","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1046\/j.0014-2956.2001.02663.x","article-title":"Domain IV of mouse laminin beta1 and beta2 chains","volume":"269","author":"Sasaki","year":"2002","journal-title":"Eur. J. Biochem."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0295","doi-asserted-by":"crossref","first-page":"9920","DOI":"10.1073\/pnas.94.18.9920","article-title":"Inactivation of the survival motor neuron gene, a candidate gene for human spinal muscular atrophy, leads to massive cell death in early mouse embryos","volume":"94","author":"Schrank","year":"1997","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0300","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":"10.1016\/j.ydbio.2011.05.667_bb0305","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":"10.1016\/j.ydbio.2011.05.667_bb0310","doi-asserted-by":"crossref","first-page":"614","DOI":"10.1038\/302614a0","article-title":"Synapse elimination in neonatal rat muscle is sensitive to pattern of muscle use","volume":"302","author":"Thompson","year":"1983","journal-title":"Nature"},{"key":"10.1016\/j.ydbio.2011.05.667_bb0315","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1113\/jphysiol.1983.sp014538","article-title":"Lack of segmental selectivity in elimination of synapses from soleus muscle of new-born rats","volume":"335","author":"Thompson","year":"1983","journal-title":"J. Physiol."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0320","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1016\/0306-4522(79)90088-5","article-title":"The effect of prolonged, reversible block of nerve impulses on the elimination of polyneuronal innervation of new-born rat skeletal muscle fibers","volume":"4","author":"Thompson","year":"1979","journal-title":"Neuroscience"},{"key":"10.1016\/j.ydbio.2011.05.667_bb0325","doi-asserted-by":"crossref","first-page":"1731","DOI":"10.1016\/S0002-9440(10)63428-1","article-title":"Deletion of murine Smn exon 7 directed to liver leads to severe defect of liver development associated with iron overload","volume":"165","author":"Vitte","year":"2004","journal-title":"Am. J. Pathol."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0330","doi-asserted-by":"crossref","first-page":"4216","DOI":"10.1093\/hmg\/ddq340","article-title":"SMN deficiency disrupts brain development in a mouse model of severe spinal muscular atrophy","volume":"19","author":"Wishart","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.ydbio.2011.05.667_bb0335","doi-asserted-by":"crossref","first-page":"538","DOI":"10.1212\/WNL.62.4.538","article-title":"Caveolinopathies: mutations in caveolin-3 cause four distinct autosomal dominant muscle diseases","volume":"62","author":"Woodman","year":"2004","journal-title":"Neurology"},{"key":"10.1016\/j.ydbio.2011.05.667_bb0340","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":"10.1016\/j.ydbio.2011.05.667_bb0345","doi-asserted-by":"crossref","first-page":"10999","DOI":"10.1523\/JNEUROSCI.3934-04.2004","article-title":"Fluorescent proteins expressed in mouse transgenic lines mark subsets of glia, neurons, macrophages, and dendritic cells for vital examination","volume":"24","author":"Zuo","year":"2004","journal-title":"J. Neurosci."}],"container-title":["Developmental Biology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0012160611009821?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0012160611009821?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,15]],"date-time":"2025-09-15T04:23:38Z","timestamp":1757910218000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0012160611009821"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,8]]},"references-count":69,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2011,8]]}},"alternative-id":["S0012160611009821"],"URL":"https:\/\/doi.org\/10.1016\/j.ydbio.2011.05.667","relation":{},"ISSN":["0012-1606"],"issn-type":[{"value":"0012-1606","type":"print"}],"subject":[],"published":{"date-parts":[[2011,8]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Muscles in a mouse model of spinal muscular atrophy show profound defects in neuromuscular development even in the absence of failure in neuromuscular transmission or loss of motor neurons","name":"articletitle","label":"Article Title"},{"value":"Developmental Biology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ydbio.2011.05.667","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2011 Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}