{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,11]],"date-time":"2026-05-11T14:44:55Z","timestamp":1778510695732,"version":"3.51.4"},"reference-count":89,"publisher":"The Company of Biologists","issue":"5","license":[{"start":{"date-parts":[[2022,6,1]],"date-time":"2022-06-01T00:00:00Z","timestamp":1654041600000},"content-version":"vor","delay-in-days":31,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"crossref","award":["R01DK117473"],"award-info":[{"award-number":["R01DK117473"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"crossref","award":["R01NS086796"],"award-info":[{"award-number":["R01NS086796"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,5,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Cardiovascular instability and a blunted respiratory drive in hypoxic conditions are hallmark features of the genetic sensory and autonomic neuropathy, familial dysautonomia (FD). FD results from a mutation in the gene ELP1, the encoded protein of which is a scaffolding subunit of the six-subunit Elongator complex. In mice, we and others have shown that Elp1 is essential for the normal development of neural crest-derived dorsal root ganglia sensory neurons. Whether Elp1 is also required for development of ectodermal placode-derived visceral sensory receptors, which are required for normal baroreception and chemosensory responses, has not been investigated. Using mouse models for FD, we here show that the entire circuitry underlying baroreception and chemoreception is impaired due to a requirement for Elp1 in the visceral sensory neuron ganglia, as well as for normal peripheral target innervation, and in their central nervous system synaptic partners in the medulla. Thus, Elp1 is required in both placode- and neural crest-derived sensory neurons, and its reduction aborts the normal development of neuronal circuitry essential for autonomic homeostasis and interoception.<\/jats:p>\n                  <jats:p>This article has an associated First Person interview with the first author of the paper.<\/jats:p>","DOI":"10.1242\/dmm.049274","type":"journal-article","created":{"date-parts":[[2022,4,28]],"date-time":"2022-04-28T06:02:09Z","timestamp":1651125729000},"source":"Crossref","is-referenced-by-count":10,"title":["<i>Elp1<\/i>\n                    is required for development of visceral sensory peripheral and central circuitry"],"prefix":"10.1242","volume":"15","author":[{"given":"Zariah","family":"Tolman","sequence":"first","affiliation":[{"name":"Montana State University 1 Department of Microbiology and Cell Biology , , Bozeman, MT 59717 , USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marta","family":"Chaverra","sequence":"additional","affiliation":[{"name":"Montana State University 1 Department of Microbiology and Cell Biology , , Bozeman, MT 59717 , USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8125-2319","authenticated-orcid":false,"given":"Lynn","family":"George","sequence":"additional","affiliation":[{"name":"Montana State University 1 Department of Microbiology and Cell Biology , , Bozeman, MT 59717 , USA"},{"name":"Montana State University Billings 2 Department of Biological and Physical Sciences , , Billings, MT 59101 , USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2064-8678","authenticated-orcid":false,"given":"Frances","family":"Lefcort","sequence":"additional","affiliation":[{"name":"Montana State University 1 Department of Microbiology and Cell Biology , , Bozeman, MT 59717 , USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"237","published-online":{"date-parts":[[2022,6,1]]},"reference":[{"key":"2022060620075608400_DMM049274C1","doi-asserted-by":"publisher","first-page":"e113428","DOI":"10.1371\/journal.pone.0113428","article-title":"Involvement of IKAP in peripheral target innervation and in specific JNK and NGF signaling in developing PNS neurons","volume":"9","author":"Abashidze","year":"2014","journal-title":"PLoS One"},{"key":"2022060620075608400_DMM049274C2","doi-asserted-by":"publisher","first-page":"1129","DOI":"10.1016\/j.cell.2019.10.031","article-title":"Genetic identification of vagal sensory neurons that control feeding","volume":"179","author":"Bai","year":"2019","journal-title":"Cell"},{"key":"2022060620075608400_DMM049274C3","doi-asserted-by":"publisher","first-page":"4524","DOI":"10.4161\/cc.22689","article-title":"A coordinated codon-dependent regulation of translation by Elongator","volume":"11","author":"Bauer","year":"2012","journal-title":"Cell Cycle"},{"key":"2022060620075608400_DMM049274C4","doi-asserted-by":"publisher","first-page":"1733","DOI":"10.1212\/01.wnl.0000335246.93495.92","article-title":"The arterial baroreflex: functional organization and involvement in neurologic disease","volume":"71","author":"Benarroch","year":"2008","journal-title":"Neurology"},{"key":"2022060620075608400_DMM049274C5","doi-asserted-by":"publisher","first-page":"452","DOI":"10.1016\/j.mcn.2010.11.010","article-title":"The formation of the cranial ganglia by placodally-derived sensory neuronal precursors","volume":"46","author":"Blentic","year":"2011","journal-title":"Mol. Cell. Neurosci."},{"key":"2022060620075608400_DMM049274C6","doi-asserted-by":"publisher","first-page":"235","DOI":"10.1016\/S0166-2236(96)01029-6","article-title":"Inadequate frameworks for understanding bodily homeostasis","volume":"20","author":"Blessing","year":"1997","journal-title":"Trends Neurosci."},{"key":"2022060620075608400_DMM049274C7","doi-asserted-by":"publisher","first-page":"131","DOI":"10.1136\/jnnp.27.2.131","article-title":"A Neuropathological study of familial dysautonomia (riley-day syndrome) in siblings","volume":"27","author":"Brown","year":"1964","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"2022060620075608400_DMM049274C8","doi-asserted-by":"publisher","first-page":"622","DOI":"10.1016\/j.cell.2015.03.022","article-title":"Vagal sensory neuron subtypes that differentially control breathing","volume":"161","author":"Chang","year":"2015","journal-title":"Cell"},{"key":"2022060620075608400_DMM049274C9","doi-asserted-by":"publisher","first-page":"310","DOI":"10.1007\/s10286-003-0131-5","article-title":"Determinants of baroreflex sensitivity in health and disease: from correlates to causality","volume":"13","author":"Chapleau","year":"2003","journal-title":"Clin. Auton. Res."},{"key":"2022060620075608400_DMM049274C10","first-page":"605","article-title":"The familial dysautonomia disease gene IKBKAP is required in the developing and adult mouse central nervous system","volume":"10","author":"Chaverra","year":"2017","journal-title":"Dis. Model. Mech."},{"key":"2022060620075608400_DMM049274C11","doi-asserted-by":"publisher","first-page":"736","DOI":"10.1128\/MCB.01313-08","article-title":"Loss of mouse Ikbkap, a subunit of elongator, leads to transcriptional deficits and embryonic lethality that can be rescued by human IKBKAP","volume":"29","author":"Chen","year":"2009","journal-title":"Mol. Cell. Biol."},{"key":"2022060620075608400_DMM049274C12","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/S0165-1838(97)00085-4","article-title":"A laser confocal microscopic study of vagal afferent innervation of rat aortic arch: chemoreceptors as well as baroreceptors","volume":"67","author":"Cheng","year":"1997","journal-title":"J. Auton. Nerv. Syst."},{"key":"2022060620075608400_DMM049274C13","doi-asserted-by":"publisher","first-page":"521","DOI":"10.1016\/j.molcel.2006.04.017","article-title":"Transcription impairment and cell migration defects in elongator-depleted cells: implication for familial dysautonomia","volume":"22","author":"Close","year":"2006","journal-title":"Mol. Cell"},{"key":"2022060620075608400_DMM049274C14","doi-asserted-by":"publisher","first-page":"663","DOI":"10.1016\/S0022-3476(55)80171-0","article-title":"Familial dysautonomia; case report with autopsy","volume":"46","author":"Cohen","year":"1955","journal-title":"J. Pediatr."},{"key":"2022060620075608400_DMM049274C15","doi-asserted-by":"publisher","first-page":"235","DOI":"10.1038\/375235a0","article-title":"Neuronal deficits, not involving motor neurons, in mice lacking BDNF and\/or NT4","volume":"375","author":"Conover","year":"1995","journal-title":"Nature"},{"key":"2022060620075608400_DMM049274C16","doi-asserted-by":"publisher","first-page":"5185","DOI":"10.1242\/dev.01383","article-title":"Ablation of TrkA function in the immune system causes B cell abnormalities","volume":"131","author":"Coppola","year":"2004","journal-title":"Development"},{"key":"2022060620075608400_DMM049274C17","doi-asserted-by":"publisher","first-page":"749","DOI":"10.1086\/368263","article-title":"Tissue-specific reduction in splicing efficiency of IKBKAP due to the major mutation associated with familial dysautonomia","volume":"72","author":"Cuajungco","year":"2003","journal-title":"Am. J. Hum. Genet."},{"key":"2022060620075608400_DMM049274C18","doi-asserted-by":"publisher","first-page":"1187","DOI":"10.1212\/WNL.0000000000003751","article-title":"Nucleus of the solitary tract, medullary reflexes, and clinical implications","volume":"88","author":"Cutsforth-Gregory","year":"2017","journal-title":"Neurology"},{"key":"2022060620075608400_DMM049274C19","doi-asserted-by":"publisher","first-page":"6635","DOI":"10.1242\/dev.00866","article-title":"Phox2b controls the development of peripheral chemoreceptors and afferent visceral pathways","volume":"130","author":"Dauger","year":"2003","journal-title":"Development"},{"key":"2022060620075608400_DMM049274C20","doi-asserted-by":"publisher","first-page":"562","DOI":"10.1016\/S0959-437X(05)80172-8","article-title":"The role of Wnt genes in vertebrate development","volume":"2","author":"Dickinson","year":"1992","journal-title":"Curr. Opin. Genet. Dev."},{"key":"2022060620075608400_DMM049274C21","doi-asserted-by":"publisher","first-page":"e27015","DOI":"10.1371\/journal.pone.0027015","article-title":"Deletion of exon 20 of the Familial Dysautonomia gene Ikbkap in mice causes developmental delay, cardiovascular defects, and early embryonic lethality","volume":"6","author":"Dietrich","year":"2011","journal-title":"PLoS One"},{"key":"2022060620075608400_DMM049274C22","doi-asserted-by":"publisher","first-page":"5361","DOI":"10.1523\/JNEUROSCI.16-17-05361.1996","article-title":"Mice lacking brain-derived neurotrophic factor exhibit visceral sensory neuron losses distinct from mice lacking NT4 and display a severe developmental deficit in control of breathing","volume":"16","author":"Erickson","year":"1996","journal-title":"J. Neurosci."},{"key":"2022060620075608400_DMM049274C23","doi-asserted-by":"publisher","first-page":"581","DOI":"10.1523\/JNEUROSCI.21-02-00581.2001","article-title":"Brain-derived neurotrophic factor and glial cell line-derived neurotrophic factor are required simultaneously for survival of dopaminergic primary sensory neurons in vivo","volume":"21","author":"Erickson","year":"2001","journal-title":"J. Neurosci."},{"key":"2022060620075608400_DMM049274C24","doi-asserted-by":"publisher","first-page":"12581","DOI":"10.1073\/pnas.0803174105","article-title":"Lack of adrenomedullin in the mouse brain results in behavioral changes, anxiety, and lower survival under stress conditions","volume":"105","author":"Fern\u00e1ndez","year":"2008","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2022060620075608400_DMM049274C25","doi-asserted-by":"publisher","first-page":"108","DOI":"10.1016\/0006-8993(92)90458-L","article-title":"The central organization of carotid body afferent projections to the brainstem of the rat","volume":"572","author":"Finley","year":"1992","journal-title":"Brain Res."},{"key":"2022060620075608400_DMM049274C88","doi-asserted-by":"publisher","first-page":"1603","DOI":"10.1038\/ijo.2013.48","article-title":"Vagal innervation patterns following Roux-en-Y gastric bypass in the mouse","volume":"37","author":"Gautron","year":"2013","journal-title":"Int J. Obes."},{"key":"2022060620075608400_DMM049274C26","doi-asserted-by":"publisher","first-page":"1287","DOI":"10.1038\/nn1962","article-title":"Nociceptive sensory neurons derive from contralaterally migrating, fate-restricted neural crest cells","volume":"10","author":"George","year":"2007","journal-title":"Nat. Neurosci."},{"key":"2022060620075608400_DMM049274C27","doi-asserted-by":"publisher","first-page":"405","DOI":"10.1002\/cne.22248","article-title":"Patterned assembly and neurogenesis in the chick dorsal root ganglion","volume":"518","author":"George","year":"2010","journal-title":"J. Comp. Neurol."},{"key":"2022060620075608400_DMM049274C28","doi-asserted-by":"publisher","first-page":"18698","DOI":"10.1073\/pnas.1308596110","article-title":"Familial dysautonomia model reveals Ikbkap deletion causes apoptosis of Pax3+ progenitors and peripheral neurons","volume":"110","author":"George","year":"2013","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2022060620075608400_DMM049274C29","doi-asserted-by":"publisher","first-page":"7319","DOI":"10.1523\/JNEUROSCI.14-12-07319.1994","article-title":"The T alpha 1 alpha-tubulin promoter specifies gene expression as a function of neuronal growth and regeneration in transgenic mice","volume":"14","author":"Gloster","year":"1994","journal-title":"J. Neurosci."},{"key":"2022060620075608400_DMM049274C30","doi-asserted-by":"publisher","first-page":"889","DOI":"10.1038\/s41467-018-03221-z","article-title":"Elongator and codon bias regulate protein levels in mammalian peripheral neurons","volume":"9","author":"Goffena","year":"2018","journal-title":"Nat. Commun."},{"key":"2022060620075608400_DMM049274C31","doi-asserted-by":"publisher","first-page":"829","DOI":"10.1152\/physrev.1994.74.4.829","article-title":"Carotid body chemoreceptors: from natural stimuli to sensory discharges","volume":"74","author":"Gonzalez","year":"1994","journal-title":"Physiol. Rev."},{"key":"2022060620075608400_DMM049274C32","doi-asserted-by":"publisher","first-page":"4141","DOI":"10.1242\/dev.02886","article-title":"Delamination of cells from neurogenic placodes does not involve an epithelial-to-mesenchymal transition","volume":"134","author":"Graham","year":"2007","journal-title":"Development"},{"key":"2022060620075608400_DMM049274C33","doi-asserted-by":"publisher","first-page":"626","DOI":"10.1016\/j.clinph.2014.06.028","article-title":"Brainstem reflexes in patients with familial dysautonomia","volume":"126","author":"Guti\u00e9rrez","year":"2015","journal-title":"Clin. Neurophysiol."},{"key":"2022060620075608400_DMM049274C34","doi-asserted-by":"publisher","first-page":"309","DOI":"10.1002\/dvdy.22527","article-title":"Epibranchial placode-derived neurons produce BDNF required for early sensory neuron development","volume":"240","author":"Harlow","year":"2011","journal-title":"Dev. Dyn."},{"key":"2022060620075608400_DMM049274C35","doi-asserted-by":"publisher","first-page":"3047","DOI":"10.1074\/jbc.M110445200","article-title":"Purification and characterization of the human elongator complex","volume":"277","author":"Hawkes","year":"2002","journal-title":"J. Biol. Chem."},{"key":"2022060620075608400_DMM049274C36","doi-asserted-by":"publisher","first-page":"34","DOI":"10.1016\/j.ydbio.2004.07.021","article-title":"Cranial sensory neuron development in the absence of brain-derived neurotrophic factor in BDNF\/Bax double null mice","volume":"275","author":"Hellard","year":"2004","journal-title":"Dev. Biol."},{"key":"2022060620075608400_DMM049274C37","doi-asserted-by":"publisher","first-page":"35","DOI":"10.1016\/j.resp.2018.01.001","article-title":"Structure of vagal afferent nerve terminal fibers in the mouse trachea","volume":"249","author":"Hennel","year":"2018","journal-title":"Respir. Physiol. Neurobiol."},{"key":"2022060620075608400_DMM049274C38","doi-asserted-by":"publisher","first-page":"424","DOI":"10.1261\/rna.7247705","article-title":"An early step in wobble uridine tRNA modification requires the Elongator complex","volume":"11","author":"Huang","year":"2005","journal-title":"RNA"},{"key":"2022060620075608400_DMM049274C39","doi-asserted-by":"publisher","first-page":"41","DOI":"10.1002\/humu.20401","article-title":"Weak definition of IKBKAP exon 20 leads to aberrant splicing in familial dysautonomia","volume":"28","author":"Ibrahim","year":"2007","journal-title":"Hum. Mutat."},{"key":"2022060620075608400_DMM049274C40","doi-asserted-by":"publisher","first-page":"2452","DOI":"10.1242\/dev.107797","article-title":"A neuron autonomous role for the familial dysautonomia gene ELP1 in sympathetic and sensory target tissue innervation","volume":"141","author":"Jackson","year":"2014","journal-title":"Development"},{"key":"2022060620075608400_DMM049274C41","doi-asserted-by":"publisher","first-page":"128","DOI":"10.1016\/j.ydbio.2005.04.004","article-title":"Mash1 is required for glomus cell formation in the mouse carotid body","volume":"283","author":"Kameda","year":"2005","journal-title":"Dev. Biol."},{"key":"2022060620075608400_DMM049274C42","doi-asserted-by":"publisher","first-page":"197","DOI":"10.1006\/dbio.2002.0689","article-title":"Homeobox gene hoxa3 is essential for the formation of the carotid body in the mouse embryos","volume":"247","author":"Kameda","year":"2002","journal-title":"Dev. Biol."},{"key":"2022060620075608400_DMM049274C43","doi-asserted-by":"publisher","first-page":"236","DOI":"10.1016\/j.ydbio.2007.12.002","article-title":"FRS2 alpha 2F\/2F mice lack carotid body and exhibit abnormalities of the superior cervical sympathetic ganglion and carotid sinus nerve","volume":"314","author":"Kameda","year":"2008","journal-title":"Dev. Biol."},{"key":"2022060620075608400_DMM049274C44","doi-asserted-by":"publisher","first-page":"1519","DOI":"10.4161\/15476286.2014.992276","article-title":"Elongator, a conserved complex required for wobble uridine modifications in eukaryotes","volume":"11","author":"Karlsborn","year":"2014","journal-title":"RNA Biol."},{"key":"2022060620075608400_DMM049274C45","doi-asserted-by":"publisher","first-page":"228","DOI":"10.1016\/j.ydbio.2016.03.015","article-title":"A cellular and molecular mosaic establishes growth and differentiation states for cranial sensory neurons","volume":"415","author":"Karpinski","year":"2016","journal-title":"Dev. Biol."},{"key":"2022060620075608400_DMM049274C46","doi-asserted-by":"publisher","first-page":"983","DOI":"10.1523\/JNEUROSCI.06-04-00983.1986","article-title":"Expression and regulation of catecholaminergic traits in primary sensory neurons: relationship to target innervation in vivo","volume":"6","author":"Katz","year":"1986","journal-title":"J. Neurosci."},{"key":"2022060620075608400_DMM049274C47","doi-asserted-by":"publisher","first-page":"3526","DOI":"10.1073\/pnas.80.11.3526","article-title":"Expression of catecholaminergic characteristics by primary sensory neurons in the normal adult rat in vivo","volume":"80","author":"Katz","year":"1983","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2022060620075608400_DMM049274C48","doi-asserted-by":"publisher","first-page":"101","DOI":"10.1016\/j.resp.2009.04.017","article-title":"Breathing disorders in Rett syndrome: progressive neurochemical dysfunction in the respiratory network after birth","volume":"168","author":"Katz","year":"2009","journal-title":"Respir. Physiol. Neurobiol."},{"key":"2022060620075608400_DMM049274C49","doi-asserted-by":"publisher","first-page":"163","DOI":"10.1056\/NEJMra1509723","article-title":"Baroreflex Dysfunction","volume":"382","author":"Kaufmann","year":"2020","journal-title":"N. Engl. J. Med."},{"key":"2022060620075608400_DMM049274C50","doi-asserted-by":"publisher","first-page":"ENEURO.0494-19.2020","DOI":"10.1523\/ENEURO.0494-19.2020","article-title":"Mapping of sensory nerve subsets within the vagal ganglia and the brainstem using reporter mice for Pirt, TRPV1, 5\u2013HT3, and Tac1 expression","volume":"7","author":"Kim","year":"2020","journal-title":"eNeuro"},{"key":"2022060620075608400_DMM049274C51","doi-asserted-by":"publisher","first-page":"2678","DOI":"10.1038\/s41467-021-22888-5","article-title":"Elp2 mutations perturb the epitranscriptome and lead to a complex neurodevelopmental phenotype","volume":"12","author":"Kojic","year":"2021","journal-title":"Nat. Commun."},{"key":"2022060620075608400_DMM049274C52","doi-asserted-by":"publisher","first-page":"1047","DOI":"10.1242\/dmm.016030","article-title":"A BDNF loop-domain mimetic acutely reverses spontaneous apneas and respiratory abnormalities during behavioral arousal in a mouse model of Rett syndrome","volume":"7","author":"Kron","year":"2014","journal-title":"Dis. Model. Mech."},{"key":"2022060620075608400_DMM049274C53","doi-asserted-by":"publisher","first-page":"331","DOI":"10.1016\/0301-0082(90)90036-G","article-title":"Arterial baroreceptor reflex: its central and peripheral neural mechanisms","volume":"35","author":"Kumada","year":"1990","journal-title":"Prog. Neurobiol."},{"key":"2022060620075608400_DMM049274C54","doi-asserted-by":"publisher","first-page":"135","DOI":"10.1007\/BF00297551","article-title":"Tyrosine-hydroxylase-containing vagal afferent neurons in the rat nodose ganglion are independent from neuropeptide-Y-containing populations and project to esophagus and stomach","volume":"271","author":"Kummer","year":"1993","journal-title":"Cell Tissue Res."},{"key":"2022060620075608400_DMM049274C55","doi-asserted-by":"publisher","first-page":"2508","DOI":"10.1016\/j.celrep.2019.04.096","article-title":"An atlas of vagal sensory neurons and their molecular specialization","volume":"27","author":"Kupari","year":"2019","journal-title":"Cell Rep."},{"key":"2022060620075608400_DMM049274C56","doi-asserted-by":"publisher","first-page":"2478","DOI":"10.1172\/JCI130401","article-title":"Retrograde nerve growth factor signaling abnormalities in familial dysautonomia","volume":"130","author":"Li","year":"2020","journal-title":"J. Clin. Invest."},{"key":"2022060620075608400_DMM049274C57","doi-asserted-by":"publisher","first-page":"930","DOI":"10.1038\/nn1299","article-title":"Neural crest boundary cap cells constitute a source of neuronal and glial cells of the PNS","volume":"7","author":"Maro","year":"2004","journal-title":"Nat. Neurosci."},{"key":"2022060620075608400_DMM049274C58","doi-asserted-by":"publisher","first-page":"949","DOI":"10.1007\/s12035-019-01782-8","article-title":"Transcriptional profiling of individual airway projecting vagal sensory neurons","volume":"57","author":"Mazzone","year":"2020","journal-title":"Mol. Neurobiol."},{"key":"2022060620075608400_DMM049274C59","doi-asserted-by":"publisher","first-page":"2192","DOI":"10.1016\/j.celrep.2019.10.040","article-title":"Arterial baroreceptors sense blood pressure through decorated aortic claws","volume":"29","author":"Min","year":"2019","journal-title":"Cell Rep."},{"key":"2022060620075608400_DMM049274C60","doi-asserted-by":"publisher","first-page":"301","DOI":"10.1016\/bs.ctdb.2014.11.009","article-title":"Transcriptional regulation of cranial sensory placode development","volume":"111","author":"Moody","year":"2015","journal-title":"Curr. Top. Dev. Biol."},{"key":"2022060620075608400_DMM049274C61","doi-asserted-by":"publisher","first-page":"1116","DOI":"10.1093\/hmg\/ddv634","article-title":"Sensory and autonomic deficits in a new humanized mouse model of familial dysautonomia","volume":"25","author":"Morini","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"2022060620075608400_DMM049274C62","doi-asserted-by":"publisher","DOI":"10.1016\/j.jgg.2021.11.011","article-title":"Developmental regulation of neuronal gene expression by Elongator complex protein 1 dosage","author":"Morini","year":"2021","journal-title":"J. Genet. Genomics"},{"key":"2022060620075608400_DMM049274C63","doi-asserted-by":"publisher","first-page":"e1006486","DOI":"10.1371\/journal.pgen.1006486","article-title":"Phosphatidylserine ameliorates neurodegenerative symptoms and enhances axonal transport in a mouse model of familial Dysautonomia","volume":"12","author":"Naftelberg","year":"2016","journal-title":"PLoS Genet."},{"key":"2022060620075608400_DMM049274C64","doi-asserted-by":"publisher","first-page":"4769","DOI":"10.1113\/jphysiol.2010.195339","article-title":"Phenotypic distinctions between neural crest and placodal derived vagal C-fibres in mouse lungs","volume":"588","author":"Nassenstein","year":"2010","journal-title":"J. Physiol."},{"key":"2022060620075608400_DMM049274C65","doi-asserted-by":"publisher","first-page":"1904","DOI":"10.1212\/WNL.0b013e3181feb283","article-title":"Afferent baroreflex failure in familial dysautonomia","volume":"75","author":"Norcliffe-Kaufmann","year":"2010","journal-title":"Neurology"},{"key":"2022060620075608400_DMM049274C66","doi-asserted-by":"publisher","first-page":"5035","DOI":"10.1073\/pnas.1620212114","article-title":"BGP-15 prevents the death of neurons in a mouse model of familial dysautonomia","volume":"114","author":"Ohlen","year":"2017","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2022060620075608400_DMM049274C67","doi-asserted-by":"publisher","first-page":"127","DOI":"10.1146\/annurev-physiol-020518-114427","article-title":"Physiology of the carotid body: from molecules to disease","volume":"82","author":"Ortega-S\u00e1enz","year":"2020","journal-title":"Annu. Rev. Physiol."},{"key":"2022060620075608400_DMM049274C68","doi-asserted-by":"publisher","first-page":"109","DOI":"10.1016\/S1097-2765(00)80179-3","article-title":"Elongator, a multisubunit component of a novel RNA polymerase II holoenzyme for transcriptional elongation","volume":"3","author":"Otero","year":"1999","journal-title":"Mol. Cell"},{"key":"2022060620075608400_DMM049274C69","doi-asserted-by":"publisher","first-page":"603","DOI":"10.1152\/jn.00808.2011","article-title":"Central chemoreceptor modulation of breathing via multipath tuning in medullary ventrolateral respiratory column circuits","volume":"107","author":"Ott","year":"2012","journal-title":"J. Neurophysiol."},{"key":"2022060620075608400_DMM049274C89","doi-asserted-by":"publisher","first-page":"2653","DOI":"10.1517\/14656566.2014.970530","article-title":"Current treatments in familial dysautonomia","volume":"15","author":"Palma","year":"2014","journal-title":"Expert. Opin. Pharmacother."},{"key":"2022060620075608400_DMM049274C70","doi-asserted-by":"publisher","first-page":"zsx083","DOI":"10.1093\/sleep\/zsx083","article-title":"Sudden unexpected death during sleep in familial dysautonomia: a case-control study","volume":"40","author":"Palma","year":"2017","journal-title":"Sleep"},{"key":"2022060620075608400_DMM049274C71","doi-asserted-by":"publisher","first-page":"10","DOI":"10.1016\/j.autneu.2019.02.003","article-title":"Chemoreflex failure and sleep-disordered breathing in familial dysautonomia: Implications for sudden death during sleep","volume":"218","author":"Palma","year":"2019","journal-title":"Auton. Neurosci."},{"key":"2022060620075608400_DMM049274C72","doi-asserted-by":"publisher","first-page":"197","DOI":"10.1038\/nn812","article-title":"Low glucose-sensing cells in the carotid body","volume":"5","author":"Pardal","year":"2002","journal-title":"Nat. Neurosci."},{"key":"2022060620075608400_DMM049274C73","doi-asserted-by":"publisher","first-page":"364","DOI":"10.1016\/j.cell.2007.07.043","article-title":"Glia-like stem cells sustain physiologic neurogenesis in the adult mammalian carotid body","volume":"131","author":"Pardal","year":"2007","journal-title":"Cell"},{"key":"2022060620075608400_DMM049274C74","doi-asserted-by":"publisher","first-page":"366","DOI":"10.1038\/20700","article-title":"The homeobox gene Phox2b is essential for the development of autonomic neural crest derivatives","volume":"399","author":"Pattyn","year":"1999","journal-title":"Nature"},{"key":"2022060620075608400_DMM049274C75","doi-asserted-by":"publisher","first-page":"252","DOI":"10.1152\/physiolgenomics.00169.2005","article-title":"Molecular profiling of murine sensory neurons in the nodose and dorsal root ganglia labeled from the peritoneal cavity","volume":"24","author":"Peeters","year":"2006","journal-title":"Physiol. Genomics"},{"key":"2022060620075608400_DMM049274C76","doi-asserted-by":"publisher","first-page":"574","DOI":"10.1016\/j.cell.2020.03.004","article-title":"An airway protection program revealed by sweeping genetic control of vagal afferents","volume":"181","author":"Prescott","year":"2020","journal-title":"Cell"},{"key":"2022060620075608400_DMM049274C77","doi-asserted-by":"publisher","first-page":"195","DOI":"10.1016\/j.cmet.2011.01.010","article-title":"Melanocortin-4 receptors expressed by cholinergic neurons regulate energy balance and glucose homeostasis","volume":"13","author":"Rossi","year":"2011","journal-title":"Cell Metab."},{"key":"2022060620075608400_DMM049274C78","doi-asserted-by":"publisher","first-page":"1803","DOI":"10.1523\/JNEUROSCI.0865-11.2012","article-title":"A TrkB small molecule partial agonist rescues TrkB phosphorylation deficits and improves respiratory function in a mouse model of Rett syndrome","volume":"32","author":"Schmid","year":"2012","journal-title":"J. Neurosci."},{"key":"2022060620075608400_DMM049274C79","doi-asserted-by":"publisher","first-page":"598","DOI":"10.1086\/318810","article-title":"Tissue-specific expression of a splicing mutation in the IKBKAP gene causes familial dysautonomia","volume":"68","author":"Slaugenhaupt","year":"2001","journal-title":"Am. J. Hum. Genet."},{"key":"2022060620075608400_DMM049274C80","doi-asserted-by":"publisher","first-page":"221","DOI":"10.1002\/dneu.22546","article-title":"The role of mitochondria in axon development and regeneration","volume":"78","author":"Smith","year":"2018","journal-title":"Dev. Neurobiol."},{"key":"2022060620075608400_DMM049274C81","doi-asserted-by":"publisher","first-page":"9725","DOI":"10.1523\/JNEUROSCI.1268-19.2019","article-title":"Contribution of the retrotrapezoid nucleus and carotid bodies to hypercapnia- and hypoxia-induced arousal from sleep","volume":"39","author":"Souza","year":"2019","journal-title":"J. Neurosci."},{"key":"2022060620075608400_DMM049274C82","doi-asserted-by":"publisher","first-page":"7649","DOI":"10.1523\/JNEUROSCI.16-23-07649.1996","article-title":"The expression pattern of the transcription factor Phox2 delineates synaptic pathways of the autonomic nervous system","volume":"16","author":"Tiveron","year":"1996","journal-title":"J. Neurosci."},{"key":"2022060620075608400_DMM049274C83","doi-asserted-by":"publisher","first-page":"ENEURO.0143-16.2016","DOI":"10.1523\/ENEURO.0143-16.2016","article-title":"Loss of Ikbkap causes slow, progressive retinal degeneration in a mouse model of familial dysautonomia","volume":"3","author":"Ueki","year":"2016","journal-title":"eNeuro"},{"key":"2022060620075608400_DMM049274C84","doi-asserted-by":"publisher","first-page":"2953","DOI":"10.1016\/j.celrep.2019.11.002","article-title":"Neurog2 deficiency uncovers a critical period of cell fate plasticity and vulnerability among neural-crest-derived somatosensory progenitors","volume":"29","author":"Vent\u00e9o","year":"2019","journal-title":"Cell Rep."},{"key":"2022060620075608400_DMM049274C85","doi-asserted-by":"publisher","first-page":"89","DOI":"10.1016\/B978-0-444-53491-0.00008-0","article-title":"Regulation of blood pressure by the arterial baroreflex and autonomic nervous system","volume":"117","author":"Wehrwein","year":"2013","journal-title":"Handb. Clin. Neurol."},{"key":"2022060620075608400_DMM049274C86","doi-asserted-by":"publisher","first-page":"209","DOI":"10.1016\/j.cell.2016.05.011","article-title":"Sensory neurons that detect stretch and nutrients in the digestive system","volume":"166","author":"Williams","year":"2016","journal-title":"Cell"},{"key":"2022060620075608400_DMM049274C87","doi-asserted-by":"publisher","first-page":"464","DOI":"10.1126\/science.aau6324","article-title":"PIEZOs mediate neuronal sensing of blood pressure and the baroreceptor reflex","volume":"362","author":"Zeng","year":"2018","journal-title":"Science"}],"container-title":["Disease Models &amp; Mechanisms"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.biologists.com\/dmm\/article-pdf\/doi\/10.1242\/dmm.049274\/2141917\/dmm049274.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/journals.biologists.com\/dmm\/article-pdf\/doi\/10.1242\/dmm.049274\/2146729\/dmm049274.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/journals.biologists.com\/dmm\/article-pdf\/doi\/10.1242\/dmm.049274\/2146729\/dmm049274.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,6,6]],"date-time":"2022-06-06T16:09:04Z","timestamp":1654531744000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.biologists.com\/dmm\/article\/15\/5\/dmm049274\/275572\/Elp1-is-required-for-development-of-visceral"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,5,1]]},"references-count":89,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2022,5,1]]}},"URL":"https:\/\/doi.org\/10.1242\/dmm.049274","relation":{"has-preprint":[{"id-type":"doi","id":"10.1101\/2022.04.11.487663","asserted-by":"object"}]},"ISSN":["1754-8403","1754-8411"],"issn-type":[{"value":"1754-8403","type":"print"},{"value":"1754-8411","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,5,1]]},"article-number":"dmm049274"}}