Abstract
Congenital birth defects, of which Hirschsprung’s disease is an example, are among the most difficult of illnesses to study in the human patients who suffer from them. By the time the condition is identified in an affected individual, the process that brought it about is over and done with. It is thus impossible to study the ontogeny of birth defects, such as Hirschsprung’s disease, in a fetus while the problems develop. An investigator seeking to uncover the pathogenesis of such a condition must search, like a detective, for clues left behind by the perpetrator who has fled the scene of a crime. Even the identification of genes that may have mutated, important an achievement as that is, does not, by itself, explain why the defect develops. Human life, moreover, is so precious that human subjects are terrible laboratory animals. As a result, more can often be learned about the origins of human illness by studying animal models, than by investigating the patients themselves. Invasive research, which is only possible on animals, can be used to develop a conceptual framework to devise hypotheses that can subsequently be tested for applicability to human patients. Experiments, based on these hypotheses, can be targeted to what can be confirmed or denied by diagnostic tests or by analyzing the restricted materials available from human subjects. Human biology is thus made approachable by knowledge of animal biology.
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References
Teitelbaum DH (1995) Hirschsprung’s disease in children. Curr Opin Pediatr 7:316–322
Larsson LT (1994) Hirschsprung’s disease — immunohistochemical findings. Histol Histopathol 9:615–629
Gershon MD, Kirchgessner AL, Wade PR (1994) Functional anatomy of the enteric nervous system. In: Johnson LR, Alpers DH, Jacobson ED, Walsh JH (eds) Physiology of the gastrointestinal tract, 3rd edn. Raven Press, New York, vol 1, pp 381–422
Furness JB, Costa M (1987) The enteric nervous system. Churchill Livingstone, New York, pp 65–69
Wood JD (1987) Physiology of the enteric nervous system. In: Johnson LR, Christensen J, Jackson MJ, Jacobson EJ, Walsh JH (eds) Physiology of the gastrointestinal tract. Raven Press, New York, vol 1, pp 67–109
Wood JD (1994) Physiology of the enteric nervous system. In: Johnson LR, Alpers DH, Jacobson ED, Walsh JH (eds) Physiology of the gastrointestinal tract, 3rd edn. Raven Press, New York, pp 423–482
Sullivan PB (1996) Hirschsprung’s disease. Arch Dis Child 74:5–7
Skinner MA (1996) Hirschsprung’s disease. Curr Probl Surg 33:389–460
Holschneider AM, Meier-Ruge W, Ure BM (1994) Hirschsprung’s disease and allied disorders — a review. Eur J Pediatr Surg 4:260–266
Qualman SJ, Murray R (1994) Aganglionosis and related disorders. Hum Pathol 25:1141–1149
Rogawski MA, Goodrich JT, Gershon MD, Touloukian RV (1978) Hirschsprung’s disease: absence of serotonergic neurons in the aganglionic colon. J Pediatric Surg 13:608–615
Teramoto M, Domoto T, Tanigawa K, Yasui Y, Tamura K (1996) Distribution of nitric oxide synthase-containing nerves in the aganglionic intestine of mutant rats: a histochemical study. J Gastroenterol 31:214–223
Tomita R, Munakata K, Kurosu Y, Tanjoh K (1995) A role of nitric oxide in Hirschsprung’s disease. J Pediatr Surg 30:437–440
Bayliss WM, Starling EH (1899) The movements and innervation of the small intestine. J Physiol (Lond) 24:99–143
Bayliss WM, Starling EH (1900) The movements and innervation of the large intestine. J Physiol (Lond) 26:107–118
Trendelenburg P (1917) Physiologische und pharmakologische Versuche über die Dünndarm Peristaltick. Naunyn-Schmiedebergs Arch Exp Pathol Pharmakol 81:55–129
Langley JN (1921) The autonomic nervous system, part 1. W Heffer, Cambridge
Gabella G (1971) Glial cells in the myenteric plexus. Z Naturforsch 26B:244–245
Gabella G (1981) Ultrastructure of the nerve plexuses of the mammalian intestine: the enteric glial cells. Neuroscience 6:425–436
Gabella G (1987) Structure of muscles and nerves of the gastrointestinal tract. In: Johnson LR, Christensen J, Jackson MJ, Jacobson EJ, Walsh JH (eds) Physiology of the gastrointestinal tract. Raven Press, New York, vol 1, pp 335–382
Gershon MD, Rothman TP (1991) Enteric glia. Glia 4:195–204
Cooke HJ (1989) Role of the “little brain” in the gut in water and electrolyte homeostasis. FASEB J 3:127–138
Crowcroft PJ, Holman ME, Szurszewski JH (1971) Excitatory input from the distal colon to the inferior mesenteric ganglion in the guinea pig. J Physiol (Lond) 219:443–461
Kreulen DL, Szurszewski JL (1979) Reflex pathways in the abdominal prevertebral ganglia: evidence for a colo-colonic inhibitory reflex. J Physiol (Lond) 295:21–32
Szurszewski JH (1981) Physiology of mammalian prevertebral ganglia. Annu Rev Physiol 43:53–68
Mawe GM, Gershon MD (1989) Relationship of gallbladder ganglia to the enteric nervous system: structure, putative neurotransmitters and direct neural connections. In: Singer MV, Goebell H (eds) Proceedings of the 50th Falk Symposium. Titisee, Germany. Kluwer Academic, pp 87–96
Kirchgessner AL, Gershon MD (1990) Innervation of the pancreas by neurons in the gut. J Neurosci 10:1626–1642
Kirchgessner AL, Gershon MD (1995) Presynaptic inhibition by serotonin (5-HT) of nerve-mediated secretion of pancreatic amylase. Am J Physiol 31:G339–G345
Scheuermann DW, Stach W (1984) Fluorescence microscopic study of the architecture and structure of an adrenergic network in the plexus myentericus (Auerbach), plexus submucosus externus (Schabadasch) and plexus submucosus internus (Meissner) of the porcine small intestine. Acta Anat 119:49–59
Timmermans J-P, Scheuermann DW, Stach W, Adriaensen D, De Groodt-Lasseel MHA (1990) Distinct distribution of CGRP-, enkephalin-. galanin-, neuromedin U-, neuropeptide Y-, somatostatin-, substance P-, VIP- and serotonin-containing neurons in the two submucosal ganglionic neural networks of the porcine small intestine. Cell Tissue Res 260:367–379
Bülbring E, Lin RCY, Schofield G (1958) An investigation of the peristaltic reflex in relation to anatomical observations. Q J Exp Physiol 43:26–37
Kirchgessner AL, Tamir H, Gershon MD (1992) Identification and stimulation by serotonin of intrinsic sensory neurons of the submucosal plexus of the guinea pig gut: activity-induced expression of Fos immunoreactivity. J Neurosci 12:235–249
Foxx-Orenstein AE, Kuemmerle JF, Grider JR (1995) The peristaltic reflex induced by mucosal stimuli in human and guinea pig intestine is mediated by distinct mucosal 5-HT receptors. Gastroenterology 108:A600
Bornstein JD, Furness JB (1988) Correlated electrophysiological and histochemical studies of submucous neurons and their contribution to understanding enteric neural circuits. J Autonom Nerv Syst 25:1–13
Frieling T, Cooke HJ, Wood JD (1991) Electrophysiological properties of neurons in submucosal ganglia of guinea pig distal colon. Am J Physiol 260:G635–G841
Jiang M-M, Kirchgessner A, Gershon MD, Surprenant A (1993) Cholera toxin-sensitive neurons in guinea pig submucosal plexus. Am J Physiol 264:G86–G94
Wattchow DA, Brookes SJH, Costa M (1995) The morphology and projections of retrogradely labeled myenteric neurons in the human intestine. Gastroenterology 109:866–875
Yntema CL, Hammond WS (1954) The origin of intrinsic ganglia of trunk viscera from vagal neural crest in the chick embryo. J Comp Neurol 101:515–542
Yntema CL, Hammond WS (1955) Experiments on the origin and development of the sacral autonomic nerves in the chick embryo. J Exp Zool 129:375–414
Le Douarin NM, Teillet MA (1974) Experimental analysis of the migration and differentiation of neuroblasts of the autonomic nervous system and of neurectodermal mesenchymal derivatives, using a biological cell marking technique. Dev Biol 41:162–184
Le Douarin NM, Teillet MA (1973) The migration of neural crest cells to the wall of the digestive tract in avian embryo. J Embryol Exp Morphol 30:31–48
Allan IJ, Newgreen DF (1980) The origin and differentiation of enteric neurons of the intestine of the fowl embryo. Am J Anat 157:137–154
Rothman TP, Gershon MD (1982) Phenotypic expression in the developing murine enteric nervous system. J Neurosci 2:381–393
Gershon MD, Chalazonitis A, Rothman TP (1993) From neural crest to bowel: development of the enteric nervous system. J Neurobiol 24:199–214
Coulter HD, Gershon MD, Rothman TP (1988) Neural and glial phenotypic expression by neural crest cells in culture: effects of control and presumptive aganglionic bowel from ls/ls mice. J Neurobiol 19:507–531
Mackey HM, Payette RF, Gershon MD (1988) Tissue effects on the expression of serotonin, tyrosine hydroxylase and GABA in cultures of neurogenic cells from the neuraxis and branchial arches. Development 104:205–217
Serbedzija GN, Burgan S, Fraser SE, Bronner-Fraser M (1991) Vital dye labeling demonstrates a sacral neural crest contribution to the enteric nervous system of chick and mouse embryos. Development 111:857–866
Pomeranz HD, Rothman TP, Gershon MD (1991) Colonization of the post-umbilical bowel by cells derived from the sacral neural crest: direct tracing of cell migration using an intercalating probe and a replication-deficient retrovirus. Development 111:647–655
Tam PKH, Lister J (1986) Development profile of neuron-specific enolase in human gut and its implications in Hirschsprung’s disease. Gastroenterology 90:1901–1906
Toyohara T, Nada O, Nagasaki A, Goto S, Ikeda K (1985) An immunohistochemical study of serotoninergic nerves in the colon and rectum of children with Hirschsprung’s disease. Acta Neuropathol 68:306–310
Durbec PL, Larsson-Blomberg LB, Schuschardt A, Costantini F, Pachnis V (1996) Common origin and developmental dependence on c-ret of subsets of enteric and sympathetic neuroblasts. Development 122:349–358
Epstein ML, Mikawa T, Brown AMC, McFarlin DR (1994) Mapping the origin of the avian enteric nervous system with a retroviral marker. Dev Dyn 201:236–244
Rothman TP, Le Douarin NM, Fontaine-Pérus JC, Gershon MD (1990) Developmental potential of neural crest-derived cells migrating from segments of developing quail bowel back-grafted into younger chick host embryos. Development 109:411–423
Rothman TP, Le Douarin NM, Fontaine-Pérus JC, Gershon MD (1993) Colonization of the bowel by neural crest-derived cells re-migrating from foregut backtransplanted to vagal or sacral regions of host embryos. Dev Dyn 196:217–233
Kapur RP, Yost C, Palmiter RD (1992) A transgenic model for studying development of the enteric nervous system in normal and aganglionic mice. Development 116:167–175
Kapur RP, Yost C, Palmiter RD (1993) Aggregation chimeras demonstrate that the primary defect responsible for aganglionic megacolon in lethal spotted mice is not neuroblast autonomous. Development 117:993–999
Coventry S, Yost C, Palmiter RD, Kapur RP (1994) Migration of ganglion cell precursors in the ileoceca of normal and lethal spotted embryos, a murine model for Hirschsprung disease. Lab Invest 71:82–93
Baetge G, Gershon MD (1989) Transient catecholaminergic (TC) cells in the vagus nerves and bowel of fetal mice: relationship to the development of enteric neurons. Dev Biol 132:189–211
Baetge G, Pintar JE, Gershon MD (1990) Transiently catecholaminergic (TC) cells in the bowel of fetal rats and mice: precursors of non-catecholaminergic enteric neurons. Dev Biol 141:353–380
Baetge G, Schneider KA, Gershon MD (1990) Development and persistence of catecholaminergic neurons in cultured explants of fetal murine vagus nerves and bowel. Development 110:689–701
Blaugrund E, Pham TD, Tennyson VM, Lo L, Sommer L, Anderson DJ, et al (1996) Distinct subpopulations of enteric neuronal progenitors defined by time of development, sympathoadrenal lineage markers, and Mash-1-dependence. Development 122:309–320
Rothman TP, Sherman D, Cochard P, Gershon MD (1986) Development of the monoaminergic innervation of the avian gut: transient and permanent expression of phenotypic markers. Dev Biol 116:357–380
Fontaine-Pérus J, Chanconie M, Le Douarin NM (1988) Developmental potentialities in the non-neuronal population of quail sensory ganglia. Dev Biol 128:359–375
Duff RS, Langtimm CJ, Richardson MK, Sieber-Blum M (1991) In vitro clonal analysis of progenitor cell patterns in dorsal root and sympathetic ganglia of the quail embryo. Dev Biol 147:451–459
Ito K, Morita T, Sieber-Blum M (1993) In vitro analysis of mouse neural crest development. Dev Biol 157:517–525
Sieber-Blum M, Cohen AM (1980) Clonal analysis of quail neural crest cells: they are pluripotent and differentiate in vitro in the absence of non-crest cells. Dev Biol 80:96–106
Baroffio A, Dupin E, Le Douarin NM (1988) Clone-forming ability and differentiation potential of migratory neural crest cells. Proc Natl Acad Sci U S A 85:5325–5329
Sextier-Sainte-Claire Deville F, Ziller C, Le Douarin N (1992) Developmental potentialities of cells derived from the truncal neural crest in clonal cultures. Dev Brain Res 66:1–10
Bronner-Fraser M, Fraser S (1989) Developmental potential of avian trunk neural crest cells in situ. Neuron 3:755–766
Bronner-Fraser M, Fraser SE (1988) Cell lineage analysis reveals multipotency of some avian neural crest cells. Nature 335:161–164
Fraser SE, Bronner-Fraser M (1991) Migrating neural crest cells in the trunk of the avian embryo are multipotent. Development 112:913–920
Sextier-Sainte-Claire Deville F, Ziller C, Le Douarin NM (1994) Developmental potentials of enteric neural crest-derived cells in clonal and mass cultures. Dev Biol 163:141–151
Lo L, Anderson DJ (1995) Postmigratory neural crest cells expressing c-RET display restricted developmental and proliferative capacities. Neuron 15:527–539
Tessier-Lavigne M (1994) Axon guidance by diffusible repellents and attractants. Curr Opin Genet Dev 4:596–601
Kennedy TE, Serafini T, de la Torre JR, Tessier-Lavigne M (1994) Netrins are diffusible chemotropic factors for commissural axons in the embryonic spinal cord. Cell 78:425–435
Serafini T, Kennedy T, Galko M, Mirzyan C, Jessell T, Tessier-Lavigne (1994) The netrins define a family of axon outgrowth-promoting proteins with homology to C. elegans UNC-6. Cell 78:409–424
Colamarino SA, Tessier-Lavigne M (1995) The axonal chemoattractant netrin-1 is also a chemorepellent for trochlear motor axons. Cell 81:621–629
Luo Y, Raible D, Raper JA (1993) Collapsin: a protein in brain that induces the collapse and paralysis of neuronal growth cones. Cell 75:217–227
Messersmith EK, Leonardo ED, Shatz CJ, Tessier-Lavigne M, Goodman CS, Kolodkin AL (1995) Semaphorin III can function as a selective repellent to pattern sensory projections in the spinal cord. Neuron 14:949–959
Kolodkin AL (1995) Semaphorins: mediators of repulsive growth cone guidance. Trends Cell Biol 61:15–22
Lallier T, Deutzmann R, Perris R, Bronner-Fraser M (1994) Neural crest cell interactions with laminin: structural requirements and localization of the binding site for alpha 1 beta 1 integrin. Dev Biol 162:451–464
Perris R, Paulsson M, Bronner-Fraser M (1989) Molecular mechanisms of neural crest cell migration on fibronectin and laminin. Dev Biol 136:222–238
Anderson DJ (1989) The neural crest cell lineage problem: neuropoiesis? Neuron 3:1–12
Le Douarin NM (1986) Cell line segregation during peripheral nervous system ontogeny. Science 231:1515–1522
Le Douarin NM, Dupin E (1993) Cell lineage analysis in neural crest ontogeny. J Neurobiol 24:146–161
Cochard P, Goldstein M, Black IB (1978) Ontogenetic appearance and disappearance of tyrosine hydroxylase and catecholamines. Proc Natl Acad Sci U S A 75:2986–2990
Teitelman G, Joh TH, Reis DJ (1978) Transient expression of a noradrenergic phenotype in cells of the rat embryonic gut. Brain Res 158:229–234
Jonakait GM, Wolf J, Cochard P, Goldstein M, Black IB (1979) Selective loss of noradrenergic phenotypic characters in neuroblasts of the rat embryo. Proc Natl Acad Sci USA 76:4683–4686
Gershon MD, Rothman TP, Joh TH, Teitelman GN (1984) Transient and differential expression of aspects of the catecholaminergic phenotype during development of the fetal bowel of rats and mice. J Neurosci 4:2269–2280
Jonakait GM, Rosenthal M, Morrell JI (1989) Regulation of tyrosine hydroxylase mRNA in the catecholaminergic cells of embryonic rat: analysis by in situ hybridization. J Histochem Cytochem 37:1–5
Rothman TP, Specht LA, Gershon MD, Joh TH, Teitelman G, Pickel VM, et al (1980) Catecholamine biosynthetic enzymes are expressed in replicating cells of the peripheral but not central nervous systems. Proc Natl Acad Sci U S A 77:6221–6225
Teitelman G, Gershon MD, Rothman TP, Joh TH, Reis DJ (1981) Proliferation and distribution of cells that transiently express a catecholaminergic phenotype during development in mice and rats. Dev Biol 86:348–355
Carnahan JF, Anderson DJ, Patterson PH (1991) Evidence that enteric neurons may derive from the sympathoadrenal lineage. Dev Biol 148:552–561
Anderson DJ, Carnahan JF, Michelsohn A, Patterson PH (1991) Antibody markers identify a common progenitor to sympathetic neurons and chromaffin cells in vivo and reveal the timing of commitment to neuronal differentiation in the sympathoadrenal lineage. J Neurosci 11:3507–3519
Johnson JE, Birren SJ, Saito T, Anderson DJ (1992) DNA binding and transcriptional regulatory activity of mammalian achaete-scute homologous (MASH) proteins revealed by interaction with a muscle-specific enhancer. Proc Natl Acad Sci U S A 89:3596–3600
Guillemot F, Joyner AL (1993) Dynamic expression of the murine achaete-scute homolog (MASH-1) in the developing nervous system. Mech Dev 42:171–185
Lo L-C, Johnson JE, Wuenschell CW, Saito T, Anderson DJ (1991) Mammalian achaete-scute homolog 1 is transiently expressed by spatially restricted subsets of early neuroepithelial and neural crest cells. Genes Dev 5:1524–1537
Guillemot F, Lo L-C, Johnson JE, Auerbach A, Anderson DJ, Joyner AL (1993) Mammalian achaete-scute homolog 1 is required for the early development of olfactory and autonomic neurons. Cell 75:463–476
Pham TD, Gershon MD, Rothman TP (1991) Time of origin of neurons in the murine enteric nervous system. J Comp Neurol 314:789–798
Pachnis V, Mankoo B, Costantini F (1993) Expression of the c-ret proto-oncogene during mouse embryogenesis. Development 119:1005–1017
Schuchardt A, D’Agati V, Larsson-Blomberg L, Costantini F, Pachnis V (1994) Defect in the kidney and enteric nervous system of mice lacking the tyrosine kinase receptor Ret. Nature 367:380–383
Jing S, Wen D, Yu Y, Holst PL, Luo Y, Fang M, et al (1996) GDNF-induced activation of the Ret protein tyrosine kinase is mediated by GDNFR-alpha, a novel receptor for GDNF. Cell 85:1113–1124
Trupp M, Arenas E, Fainzilber M, Nilsson A-S, Sieber B-A, Grigoriou M, et al (1996) Functional receptor for GDNF encoded by the c-ret proto-oncogene. Nature 381:785–789
Durbec P, Marcos-Gutierrez CV, Kilkenny C, Grigoriou M, Wartiowaara K, Suvanto P, et al (1996) GDNF signalling through the Ret receptor tyrosine kinase. Nature 381:789–793
Lin L-FH, Doherty DH, Lile JD, Bektesh S, Collins F (1993) GDNF: a glial cell line-derived neurotrophic factor for midbrain dopinergic neurons. Science 260:1130–1132
Trupp M, Rydén M, Jörnvall H, Funakoshi H, Timmusk T, Arenas E, et al (1995) Peripheral expression and biological activities of GDNF, a new neurotrophic factor for avian and mammalian peripheral neurons. J Cell Biol 130:137–148
Choi-Lundberg DL, Bohn MC (1995) Ontogeny and distribution of glial cell line-derived neurotrophic factor. Brain Res Dev Brain Res 85:80–88
Tsuzuki T, Takahashi M, Asai N, Iwashita T, Matsuyama M, Asai J (1995) Spatial and temporal expression of the ret proto-oncogene product in embryonic, infant, and adult rat tissues. Oncogene 10:191–198
Sénchez M, Silos-Santiago I, Frisén J, He B, Lira S, Barbacid M (1996) Renal agenesis and the absence of enteric neurons in mice lacking GDNF. Nature 382:70–73
Moore MW, Klein RD, Fariñas I, Sauer H, Armanini M, Phillips H, et al (1996) Renal and neuronal abnormalities in mice lacking GDNF. Nature 382:76–79
Pichel JG, Shen L, Sheng HZ, Granholm A-C, Drago J, Grinberg A, et al (1996) Defects in enteric innervation and kidney development in mice lacking GDNF. Nature 382:73–76
Treanor JJS, Goodman L, de Sauvage F, Stone DM, Poulsen KT, Beck CD, et al (1996) Characterization of a multicomponent receptor for GDNF. Nature 382:80–83
Dreyfus CF, Bornstein MB, Gershon MD (1977) Synthesis of serotonin by neurons of the myenteric plexus in situ and in organotypic tissue culture. Brain Res 128:125–139
Dreyfus CF, Sherman D, Gershon MD (1977) Uptake of serotonin by intrinsic neurons of the myenteric plexus grown in organotypic tissue culture. Brain Res 128:109–123
Johnson EMJ, Osborne P, Rydel RE, Schmidt RE, Pearson J (1983) Characterization of the effects of autoimmune nerve growth factor deprivation in the developing guinea-pig. Neuroscience 8:631–642
Pearson J, Johnson EM, Brandeis L (1983) Effects of antibodies to nerve growth factor on intrauterine development of derivatives of cranial neural crest and placode in the guinea pig. Dev Biol 96:32–36
Gershon MD, Rothman TP, Sherman D, Johnson EM (1983) Effect of prenatal exposure to anti-NGF on the enteric nervous system (ENS) of the guinea pig. Anat Rec 205:62A
Barbacid M (1993) The trk family of neurotrophin receptors: molecular characterization and oncogenic activation in human tumors. In: Levine AJ, Schmidek HH (eds) Molecular genetics of nervous system tumors. Wiley & Sons, New York, pp 123–135
Chao MV, Hempstead BL (1995) p75 and Trk: a two-receptor system. Trends Neurosci 18:321–326
Lindsay RM, Wiegand SJ, Altar CA, DiStefano PS (1994) Neurotrophic factors: from molecule to man. Trends Neurosci 17:182–190
Götz R, Koster R, Winkler C, Raulf F, Lottspeich F, Schartl M, et al (1994) Neurotrphin-6 is a new member of the nerve growth factor family. Nature 372:266–269
Bothwell M (1996) P75NTR: a receptor after all. Science 272:506–507
Carter BD, Kaltschmidt C, Kaltschmidt B, Offenhäuser N, Böhm-Matthaei R, Baeuerle PA, et al (1996) Selective activation of NFkB by nerve growth factor through the neurotrophin receptor p75. Science 272:542–545
Tsoulfas P, Soppet D, Escandon E, Tessarollo L, Mandoza-Ramirez J-L, Rosenthal A, et al (1993) The rat trkC locus encodes multiple neurogenic receptors that exhibit differential response to neurotrophin-3 in PC12 cells. Neuron 10:975–990
Pomeranz HD, Rothman TP, Chalazonitis A, Tennyson VM, Gershon MD (1993) Neural crest-derived cells isolated from the gut by immunoselection develop neuronal and glial phenotypes when cultured on laminin. Dev Biol 156:341–361
Chalazonitis A, Rothman TP, Chen J, Lamballe F, Barbacid M, Gershon MD (1994) Neurotrophin-3 induces neural crest-derived cells from fetal rat gut to develop in vitro as neurons or glia. J Neurosci 14:6571–6584
Verdi JM, Anderson DJ (1994) Neurotrophins regulate sequential changes in neurotrophin receptor expression by sympathetic neuroblasts. Neuron 13:1359–1372
Birren SJ, Lo L, Anderson DJ (1993) Sympathetic neuroblasts undergo a developmental switch in trophic dependence. Development 119:597–610
DiCicco-Bloom E, Friedman WJ, Black IB (1993) NT-3 stimulates sympathetic neuroblast proliferation by promoting precursor survival. Neuron 11:1101–1111
Black IB (1978) Regulation of autonomic development. Annu Rev Neurosci 1:183–214
Wyatt S, Davies AM (1995) Regulation of nerve growth factor receptor gene expression in sympathetic neurons during development. J Cell Biol 130:1–12
Zhang JM, Winslow JW, Sieber-Blum M (1993) Role of neurotrophin-3 (NT-3) in the expression of the adrenergic phenotype by neural crest cells. Neuroscience Abstract 19:251
Ernfors P, Lee K-F, Kucera J, Jaenisch R (1994) Lack of neurotrophin-3 leads to deficiencies in the peripheral nervous system and loss of limb proprioceptive afferents. Cell 77:503–512
Fariñas I, Jones KR, Backus C, Wang X-Y, Reichardt LF (1994) Severe sensory and sympathetic deficits in mice lacking neurotrophin-3. Nature 369:658–661
Zhou X-F, Rush RA (1995) Sympathetic neurons in neonatal rats require endogenous neurotrophin-3 for survival. J Neurosci 15:6521–6530
ElShamy WM, Linnarsson S, Lee K-F, Jaenisch R, Ernfors P (1996) Prenatal and postnatal requirements of NT-3 for sympathetic neuroblast survival and innervation of specific targets. Development 122:491–500
Lamballe F, Smeyne R, Barbacid M (1994) Developmental expression of TrkC, the neurotrophin-3 receptor in the mammalian nervous system. J Neurosci 14:14–28
Tessarollo L, Tsoulfas P, Martin-Zanca D, Gilbert DJ, Jenkins NA, Copeland NG, et al (1993) trkC, a receptor for neurotrophin-3, is widely expressed in the developing nervous and in non-neuronal tissues. Development 118:463–475
Escandón E, Soppet D, Rosenthal A, Mendoza-Ramirez J-L, Szönyi É, Burton LE, et al (1994) Regulation of neurotrophin receptor expression during embryonic and postnatal development. J Neurosci 14:2054–2068
Tojo H, Kaisho Y, Nakata M, Matsuoka K, Kitagawa M, Abe T, et al (1995) Targeted disruption of the neurotrophin-3 gene with lacZ induces loss of trkC-positive neurons in sensory ganglia but not in spinal cords. Brain Res 669:163–175
Payette RF, Bennett GS, Gershon MD (1984) Neurofilament expression in vagal neural crest-derived precursors of enteric neurons. Dev Biol 105:273–287
Payette RF, Tennyson VM, Pham TD, Mawe GM, Pomeranz HD, Rothman TP, et al (1987) Origin and morphology of nerve fibers in the aganglionic colon of the lethal spotted (ls/ls) mutant mouse. J Comp Neurol 257:237–252
Chalazonitis A, Rothman TP, Gershon MD (1995) Ciliary neurotrophic factor (CNTF) and neurotrophin-3 (NT-3) potentiate one another in promoting the enteric neuronal development. Neuroscience Abstract 25:1545
Chalazonitis A (1996) Neurotrophin-3 as an essential signal for the developing nervous system. Mol Neurobiol 12:39–53
Ockel M, Lewin GR, Barde Y-A (1996) In vivo effects of neurotrphin-3 during sensory neurogenesis. Development 122:301–307
Kalcheim C, Carmeli C, Rosenthal A (1992) Neurotrophin-3 is a mitogen for cultured neural crest cells. Proc Natl Acad Sci U S A 89:1661–1665
Pinco O, Carmeli C, Rosenthal A, Kalcheim C (1993) Neurotrophin-3 affects proliferation and differentiation of distinct neural crest cells and is present in the early neural tube of avian embryos. J Neurobiol 24:1626–1641
Pham T, Wade A, Chalazonitis A, Skirboll SL, Bothwell M, Gershon MD (1996) Increased numbers of myenteric neurons arise in transgenic mice that overexpress neurotrophin-3 (NT-3) directed to the enteric nervous system (ENS) by the dopamine beta-hydroxylase promoter. Neuroscience Abstract 12:999
Klein R, Silos-Santiago I, Smeyne RJ, Lira SA, Brambilla R, Bryant S, et al (1994) Disruption of the neurotrophin-3 receptor gene trkC eliminates 1a muscle afferents and results in abnormal movements. Nature 368:249–251
Lee K-F, Li E, Huber LJ, Landis S, Sharpe AH, Chao MV, et al (1992) Targeted mutation of the gene encoding the low affinity NGF receptor p75 leads to deficits in the peripheral sensory nervous system. Cell 69:737–749
Henderson CE (1996) Role of neurotrophic factors in neuronal development. Curr Opin Neurobiol 6:64–70
Adler R, Landa KB, Manthorpe M, Varon S (1979) Cholinergic neuronotrophic factors: intraocular distribution of soluble trophic activity for ciliary neurons. Science 204:1434–1436
Sendtner M, Carroll P, Holtmann B, Hughes RA, Thoenen H (1994) Ciliary neurotrophic factor. J Neurobiol 25:1436–1453
DeChiara TM, Vejsada R, Poueymirou WT, Acheson A, Suri C, Conover JC, et al (1995) Mice lacking the CNTF receptor, unlike mice lacking CNTF, exhibit profound motor neuron deficits at birth. Cell 83:313–322
Pennica D, Shaw KJ, Swanson TA, Moore MW, Shelton DL, Zioncheck KA, et al (1995) Cardiotrophin-1. Biological activities and binding to the leukemia inhibitory factor receptor/gp130 signaling complex. J Biol Chem 270:10915–10922
Davis S, Aldrich TH, Valenzuela D, Wong V, Furth ME, Squinto SP, et al (1991) The receptor for ciliary neurotrophic factor. Science 253:59–63
Gearing DP, Thut CJ, VandenBos T, Gimpel SD, Delaney PB, King J, et al (1991) Leukemia inhibitory factor receptor is structurally related to the IL-6 signal transducer, gp130. EMBO J 10:2839–2848
Gearing DP, Comeau MR, Friend DJ, Gimpel SD, Thust CJ, McGourty J, et al (1992) The Il-6 signal transducer, gp130: an oncostatin M receptor and affinity converter for the LIF receptor. Science 255:1434–1437
Ip NY, Nye SH, Boulton TG, Davis S, Taga T, Li Y, et al (1992) CNTF and LIF act on neuronal cells via shared signalling pathways that involve the IL-6 signal transducing receptor component gp130. Cell 69:1121–1132
Davis S, Aldrich TH, Stahl N, Taga T, Kishimoto T, Ip NY, et al (1993) LIFRb and gp130 as heterodimerizing signal transducers in the tripartite CNTF receptor. Science 260:1805–1808
Kishimoto T, Taga T, Akira S (1994) Cytokine signal transduction. Cell 76:253–262
Stahl N, Boulton TG, Farruggella T, Ip NY, Davis S, Witthuhn B, et al (1994) Association and activation of Jak-Tyk kinases by CNTF-LIF-OSM-IL6b receptor components. Science 263:92–95
Rothman TP, Chen J, Gershon MD (1994) Microenvironmental factors in the differentiation of enteric neurons from neural crest-derived precursors. Neuroscience Abstract 20:1492
Ip NY, McClain J, Barrezueta NX, Aldrich TH, Pan L, Li Y, et al (1993) The alpha component of the CNTF receptor is required for signalling and defines potential CNTF targets in the adult and during development. Neuron 10:89–102
Masu Y, Wold E, Holtmann BS, M, Brem G, Thoenen H (1993) Disruption of the CNTF gene results in motor neuron degeneration. Nature 365:27–32
Takahashi R, Yokoji H, Misawa H, Hayashi M, Hu J, Deguchi T (1994) A null mutation in the human CNTF gene is not causally related to neurological diseases. Nat Genet 7:79–84
Brookes SJH, Steele PA, Costa M (1991) Identification and immunohistochemistry of cholinergic and non-cholinergic circular muscle motor neurons in the guinea-pig small intestine. Neuroscience 42:863–878
Bult H, Boeckxstaens GE, Pelckmans PA, Jordaens FH, van Maercke YM, Herman AG (1990) Nitric oxide as an inhibitory non-adrenergic non-cholinergic neurotransmitter. Nature 345:346–347
Costa M, Furness JB, Pompolo S, Brookes SJH, Bornstein JC, Bredt DS, et al (1992) Projections and chemical coding of neurons with immunoreactivity for nitric oxide synthase in the guinea-pig small intestine. Neurosci Lett 148:121–125
Konturek SJ, Bilski J, Konturek PK, Cieszkowski M, Pawlik W (1993) Role of endogenous nitric oxide in the control of canine pancreatic secretion and blood flow. Gastroenterology 104:896–902
Stark ME, Bauer AJ, Szurszewski JH (1991) Effect of nitric oxide on circular muscle of the canine small intestine. J Physiol (Lond) 444:743–761
Young HM, McConalogue K, Furness JB, De Vente J (1993) Nitric oxide targets in the guinea-pig intestine identified by induction of cyclic GMP immunoreactivity. Neuroscience 55:583–596
Ernsberger U, Sendtner M, Rohrer H (1989) Proliferation and differentiation of embryonic chick sympathetic neurons: effects of ciliary neurotrophic factor. Neuron 2:1275–1284
Saadat S, Sendtner M, Rohrer H (1989) Ciliary neurotrophic factor induces cholinergic differentiation of rat sympathetic neurons in culture. J Cell Biol 108:1807–1816
Lane PW (1966) Association of megacolon with two recessive spotting genes in the mouse. J Hered 57:29–31
Bolande RP (1975) Animal model of human disease. Hirschsprung’s disease, aganglionic or hypoganglionic megacolon: animal model: aganglionic megacolon in piebald and spotted mutant mouse strains. Am J Pathol 79:189–192
Watanabe Y, Ito T, Harada T, Kobayashi S, Ozaki T, Nimura Y (1995) Spatial distribution and pattern of extrinsic nerve strands in the aganglionic segment of congenital aganglionosis: stereoscopic analysis in spotting lethal rats. J Pediatr Surg 30:1471–1476
Karaki H, Mitsui-Saito M, Takimoto M, Oda K, Okada T, Ozaki, et al (1996) Lack of endothelin ETB receptor binding and function in the rat with a mutant ETB receptor gene. Biochem Biophys Res Commun 222:139–143
Watanabe Y, Ito T, Harada T, Takahashi M, Kobayashi S, Ozaki T, et al (1995) Expression of ret proto-oncogene products in the hypoganglionic segment of the small intestine of congenital aganglionosis rats. J Pediatr Surg 30:641–645
Ceccherini I, Zhang A, Matera I, Yang G, Devoto M, Romeo G, et al (1995) Interstitial deletion of the endothelin-B receptor gene in the spotting lethal (sl) rat. Hum Mol Genet 4:2089–2096
Gariepy CE, Cass DT, Yanagisawa M (1996) Null mutation of endothelin receptor type B gene in spotting lethal rats causes aganglionic megacolon and white coat color. Proc Natl Acad Sci U S A 93:867–872
Bodeker D, Turck O, Loven E, Wieberneit D, Wegner W (1995) Pathophysiological and functional aspects of the megacolon-syndrome of homozygous spotted rabbits. Zentralbl Veterinarmed A 42:549–559
Poirier V, Goossens M, Simonneau M (1996) Neuronal defects in genotyped dominant megacolon (Dom) mouse embryos, a model for Hirschsprung disease. Neuroreport 7:489–492
Kapur RP, Livingston R, Doggett B, Sweetser DA, Siebert JR, Palmiter RD (1996) Abnormal microenvironmental signals underlie intestinal aganglionosis in Dominant megacolon mutant mice. Dev Biol 174:360–369
Baynash AG, Hosoda K, Giaid A, Richardson JA, Emoto N, Hammer RE, et al (1994) Interaction of endothelin-3 with endothelin-B receptor is essential for development of epidermal melanocytes and enteric neurons. Cell 79:1277–1285
Hosoda K, Hammer RE, Richardson JA, Baynash AG, Cheung JC, Giaid A, et al (1994) Targeted and natural (piebald-lethal) mutation of endothelin-B receptor produce megacolon associated with spotted coat color in mice. Cell 79:1267–1276
Puffenberger EG, Hosoda K, Washington SS, Nakao K, deWit D, Yanagisawa M, et al (1994) A missense mutation of the endothelin-receptor gene in mutagenic Hirschsprung’s disease. Cell 79:1257–1266
Rubanyi GM, Polokoff MA (1994) Endothelins: molecular biology, biochemistry, pharmacology, physiology, and pathophysiology. Pharmacol Rev 46:325–415
Sakamoto A, Yanagisawa M, Sawamura T, Enoki T, Ohtani T, Sakurai T, et al (1993) Distinct subdomains of human endothelin receptors determine their selectivity to ETA-selective antagonist and ETB-selective agonists. J Biol Chem 268:8547–8553
Yanagisawa M (1994) The endothelin system: a new target for therapeutic intervention. Circulation 89:1320–1322
Yanagisawa M, Kurihara H, Kimura S, Tomobe Y, Kobayshi M, Mitsui Y, et al (1988) A novel potent vasoconstrictor peptide produced by vascular endothelial cells. Nature 332:411–415
Xu D, Emoto J, Giaid A, Slaughter C, Kaw S, deWit D, et al (1994) ECE-1: a membrane-bound metalloprotease that catalyzes the proteolytic activation of big endothelin-1. Cell 78:473–485
Kurihara Y, Kurihara H, Suzuki H, Kodama T, Maemura K, Nagai RO, H, et al (1994) Elevated blood pressure and craniofacial abnormalities in mice deficient in endothlin-1. Nature 368:703–710
Kapur RP, Sweetser DA, Doggett B, Siebert JR, Palmiter RD (1995) Intercellular signals downstream of endothelin receptor-B mediate colonization of the large intestine by enteric neuroblasts. Development 121:3787–3795
Lahav R, Ziller C, Dupin E, Le Douarin NM (1996) Endothelin 3 promotes neural crest cell proliferation and mediates a vast increase in melanocyte number in culture. Proc Natl Acad Sci U S A 93:3892–3897
Rothman TP, Goldowitz D, Gershon MD (1993) Inhibition of migration of neural crest-derived cells by the abnormal mesenchyme of the presumptive aganglionic bowel of ls/ls mice: analysis with aggregation and interspecies chimeras. Dev Biol 159:559–573
Rothman TP, Gershon MD (1984) Regionally defective colonization of the terminal bowel by the precursors of enteric neurons in lethal spotted mutant mice. Neuroscience 12:1293–1311
Rothman TP, Tennyson VM, Gershon MD (1986) Colonization of the bowel by the precursors of enteric glia: studies of normal and congenitally aganglionic mutant mice. J Comp Neurol 252:493–506
Jacobs-Cohen RJ, Payette RF, Gershon MD, Rothman TP (1987) Inability of neural crest cells to colonize the presumptive aganglionic bowel of ls/ls mutant mice: requirement for a permissive microenvironment. J Comp Neurol 255:425–438
Payette RF, Tennyson VM, Pomeranz HD, Pham TD, Rothman TP, Gershon MD (1988) Accumulation of components of basal laminae: association with the failure of neural crest cells to colonize the presumptive aganglionic bowel of ls/ls mutant mice. Dev Biol 125:341–360
Tennyson VM, Payette RF, Rothman TP, Gershon MD (1990) Distribution of hyaluronic acid and chondroitin sulfate proteoglycans in the presumptive aganglionic terminal bowel of ls/ls fetal mice: an ultrastructural analysis. J Comp Neurol 291:345–362
Rothman TP, Chen J, Howard MJ, Costantini FD, Pachnis V, Gershon MD (1996) Increased expression of laminin-1 and collagen (IV) subunits in the aganglionic bowel of ls/ls, but not c-ret −/− mice. Dev Biol 178:498–513
Parikh DH, Tam PKH, VanVelzen D, Edgar D (1992) Abnormalities in the distribution of laminin and collagen type IV in Hirschsprung’s disease. Gastroenterology 102:1236–1241
Parikh DH, Leibl M, Tam PK, Edgar D (1995) Abnormal expression and distribution of nidogen in Hirschsprung’s disease. J Pediatr Surg 30:1687–1693
Tucker GC, Ciment G, Thiery J-P (1986) Pathways of avian neural crest cell migration in the developing gut. Dev Biol 116:439–450
Pomeranz HD, Gershon MD (1990) Colonization of the avian hindgut by cells derived from the sacral neural crest. Dev Biol 137:378–394
Pomeranz HD, Sherman DL, Smalheiser NR, Tennyson VM, Gershon MD (1991) Expression of a neurally related laminin binding protein by neural crest-derived cells that colonize the gut: relationship to the formation of enteric ganglia. J Comp Neurol 313:625–642
Oakley RA, Lasky CJ, Erickson CA, Tosney KW (1994) Glycoconjugates mark a transient barrier to neural crest migration in the chicken embryo. Development 120:103–114
Erickson CA, Duong TD, Tosney KW (1992) Descriptive and experimental analysis of the dispersion of neural crest cells along the dorsolateral path and their entry into ectoderm in the chick embryo. Dev Biol 151:251–272
Rickmann M, Fawcett JW, Keynes RJ (1985) The migration of neural crest cells and the growth of motor axons through the rostral half of the chick somite. J Embryol Exp Morphol 90:437–455
Norris WE, Stern CD, Keynes RJ (1989) Molecular differences between the rostral and caudal halves of the sclerotome in the chick embryo. Development 105:541–548
Keynes RJ, Johnson AR, Picart CJ, Dunin-Borkowski OM, Cook GMW (1990) A glycoprotein fraction from adult chicken grey matter causes collapse of CNS and PNS growth cones in vitro. Neuroscience Abstract 16:169
Pettway Z, Guillory G, Bronner-Fraser M (1990) Absence of neural crest cells from the region surrounding notochords in situ. Dev Biol 142:335–345
Oakley RA, Tosney KW (1991) Peanut agglutinin and chrondroitin-6-sulfate are molecular markers for tissues that act as barrier to axon advance in the avian embryo. Dev Biol 147:187–206
Miura H, Ohi R, Tseng SW, Takahashi T (1996) The structure of the transitional and aganglionic zones of Auerbach’s plexus in patients with Hirschsprung’s disease: a computer-assisted three-dimensional reconstruction study. J Pediatr Surg 31:420–426
Lallier T, Bronner-Fraser M (1991) Avian neural crest cell attachment to laminin: involvement of divalent cation dependent and independent integrins. Development 113:1069–1084
Bilozur ME, Hay ED (1988) Neural crest migration in 3D extracellular matrix utilizes laminin, fibronectin, or collagen. Dev Biol 125:19–33
Bronner-Fraser M (1985) Alterations in neural crest migration by a monoclonal antibody that affects cell adhesion. J Cell Biol 101:610–617
Bronner-Fraser M (1986) An antibody to a receptor for fibronectin and laminin perturbs cranial neural crest development in vivo. Dev Biol 117:528–536
Bronner-Fraser M, Lallier T (1988) A monoclonal antibody against a laminin-heparan sulfate proteoglycan complex perturbs cranial neural crest migration in vivo. J Cell Biol 106:1321–1329
Engvall E, Davis GE, Dickerson K, Ruoslahti E, Varon S, Manthorpe M (1986) Mapping of domains in human laminin using monoclonal antibodies: localization of the neurite-promoting site. J Cell Biol 103:2457–2465
Kleinman HK, Ogle RC, Cannon FB, Little CD, Sweeney TM, Luckenbill-Edds L (1988) Laminin receptors for neurite formation. Proc Natl Acad Sci U S A 85:1282–1286
Lander AD, Fujii DK, Reichardt LF (1985) Laminin is associated with the neurite outgrowth-promoting factors found in conditioned media. Proc Natl Acad Sci U S A 82:2183–2187
Liesi P, Narvanen A, Soos J, Sariola H, Snounou G (1989) Identification of a neurite outgrowth-promoting domain of laminin using synthetic peptides. FEBS Lett 244:141–148
Manthorpe M, Engvall E, Ruoslahti E, Longo FM, Davis GE, Varon S (1983) Laminin promotes neuritic regeneration from cultured peripheral and central neurons. J Cell Biol 97:1882–1890
Calof AL, Reichardt LF (1985) Response of purified chick motoneurons to myotube conditioned medium: laminin is essential for the substratum-binding neurite outgrowth-promoting activity. Neurosci Lett 59:183–189
Stemple D, Anderson DJ (1992) Isolation of a stem cell for neurons and glia from the mammalian neural crest. Cell 71:973–985
Gershon MD, Chalazonitis A, Blaugrund E, Tennyson VM, Rothman TP (1993) Laminin and neurotrophin-3 (NT-3) in the formation of the enteric nervous system. Anat Rec Suppl 1:54
Tennyson VM, Chalazonitis A, Kibbey MC, Gershon MD (1995) Laminin-1 stimulates a cell surface receptor (LBP110) to promote the differentiation of enteric neurons in vitro independently of its role as an adhesion molecule. Neuroscience Abstract 21:788
Chalazonitis A, Tennyson VM, Rothman TP, Gershon MD (1992) Selective isolation of neural and glial precursors from the developing murine bowel with antibodies to a 110 kDa cell surface laminin binding protein. Neuroscience Abstract 18:1109
Douville PJ, Harvey WJ, Carbonetto S (1988) Isolation and partial characterization of high affinity laminin receptors in neural cells. J Biol Chem 263:14964–14969
Kleinman HK, Weeks BS (1989) Laminin: structure functions and receptors. Curr Sci 1:964–967
Jucker M, Kleinman HK, Walker LC, Bialobok P, Williams LR, Ingram DK (1991) Localization and characterization of a 110 KD laminin binding protein immunoreactivity in adult and lesioned brain. Neuroscience Abstract 17:207
Kibbey MC, Jucker M, Weeks BS, Neve RL, VanNostrand WE, Kleinman HK (1993) beta-Amyloid precursor protein binds to the neurite-promoting IKVAV site of laminin. Proc Natl Acad Sci U S A 90:10150–10153
Kleinman HK, Weeks BS, Cannon FB, Sweeney TM, Sephel GC, Clement B, et al (1991) Identification of a 110 Kd nonintegrin cell surface laminin-binding protein which recognizes an A chain neurite-promoting peptide. Arch Biochem Biophys 290:320–325
Sephel GC, Tashiro K, Sasaki M, Kandel S, Yamada Y, Kleinman HK (1989) A laminin-pepsin fragment with cell attachment and neurite outgrowth activity at distinct sites. Dev Biol 135:172–181
Sephel GC, Tashiro K, Kleinman HK, Sasaki M, Yamada Y, Martin GR (1989) A laminin A chain synthetic peptide with neurite outgrowth activity. Biochem Biophys Res Commun 162:821–829
Bresalier RS, Schwartz B, Kim YS, Duh QY, Kleinman HK, Sullam PM (1995) The laminin alpha 1 chain Ile-Lys-Val-Ala-Val (IKVAV)-containing peptide promotes liver colonization by human colon cancer cells. Cancer Res 55:2476–2480
Corcoran ML, Kibbey MC, Kleinman HK, Wahl LM (1995) Laminin SIKVAV peptide induction of monocyte/macrophage prostaglandin E2 and matrix metalloproteinases. J Biol Chem 270:10365–10368
Haralabopoulos GC, Grant DS, Kleinman HK, Lelkes PI, Papaioannou SP, Maragoudakis ME (1994) Inhibitors of basement membrane collagen synthesis prevent endothelial cell alignment in matrigel in vitro and angiogenesis in vivo. Lab Invest 71:575–582
Kibbey MC, Corcoran ML, Wahl LM, Kleinman HK (1994) Laminin SIKVAV peptide-induced angiogenesis in vivo is potentiated by neutrophils. J Cell Physiol 160:185–193
Nomizu M, Weeks BS, Weston CA, Kim WH, Kleinman HK, Yamada Y (1995) Structure-activity study of a laminin alpha 1 chain active peptide segment Ile-Lys-Val-Ala-Val (IKVAV). FEBS Lett 365:227–231
Weeks BS, Holloway E, Klotman PE, Akiyama SK, Schnaper HW, Kleinman HK (1994) 12-O-tetradecanoylphorbol 13-acetate stimulates human T-lymphocyte adherence to the fibronectin RGD domain and the laminin IKVAV domain. Cell Immunol 153:94–104
Stemple DL, Anderson DJ (1993) Lineage diversification of the neural crest: in vitro investigations. Dev Biol 159:12–23
Erickson CA, Loring JF, Lester SM (1989) Migratory pathways of HNK-1-immunoreactive neural crest cells in the rat embryo. Dev Biol 134:112–118
Martins-Green M, Erickson CA (1987) Basal lamina is not a barrier to neural crest emigration: documentation by TEM and by immunofluorescent and immunogold labelling. Development 101:517–533
Okabe H, Chijiiwa Y, Nakamura K, Yoshinaga M, Akihi H, Harada N, et al (1995) Two endothelin receptors (ETA and ETB) expressed on circular smooth muscle cells of guinea pig cecum. Gastroenterology 108:51–57
Yoshinaga M, Chijiiwa Y, Misawa T, Harada N, Nawata H (1992) Endothelin-B receptor on guinea small intestinal smooth muscle cells. Am J Physiol 25:G308–G311
Vanderwinden JM, Rumessen JJ, Liu H, Descamps D, De Laet MH, Vanderhaeghen JJ (1996) Interstitial cells of Cajal in human colon and in Hirschsprung’s disease. Gastroenterology 111:901–910
Yamataka A, Kato Y, Tibboel D, Murata Y, Sueyoshi N, Fujimoto T, et al (1995) A lack of intestinal pacemaker (c-kit) in aganglionic bowel of patients with Hirschsprung’s disease. J Pediatr Surg 30:441–444
Torihashi S, Ward SM, Sanders KM (1997) Development of c-kit-positive cells and the onset of electrical rhythmicity in murine small intestine. Gastroenterology 112:144–155
Kobayashi S, Furness JB, Smith TK, Pompolo S (1989) Histological identification of the interstitial cells of Cajal in the guinea-pig small intestine. Arch Histol Cytol 52:267–286
Cook RD, Burnstock G (1976) The ultrastructure of Auerbach’s plexus in the guinea-pig. II. Non-neuronal elements. J Neurocytol 5:195–206
Faussone-Pellegrini MS (1985) Cytodifferentiation of the interstitial cells of Cajal related to the myenteric plexus of mouse intestinal coat. Acta Embryol 171:163–169
Torihashi S, Kobayashi S, Gerthoffer WT, Sanders KM (1993) Interstitial cells in deep muscular plexus of canine small intestine may be specialized smooth muscle cells. Am J Physiol 265:G638–G645
Ward SM, Burns AJ, Torihashi S, Sanders KM (1994) Mutation of the proto-oncogene c-kit blocks development of interstitial cells and electrical rhythmicity in murine intestine. J Physiol 480:91–97
Huizinga JD, Thuneberg L, Klüppel M, Malysz J, Mikkelsen HB, Bernstein A (1995) W/kit gene required for interstitial cells of Cajal and for intestinal pacemaker activity. Nature 373:347–349
Torihashi S, Ward SM, Nishikawa S, Nishi K, Kobayashi S, Sanders KM (1995) c-kit-dependent development of interstitial cells and electrical activity in the murine gastrointestinal tract. Cell Tissue Res 280:97–111
Ward SM, Burns AJ, Torishashi S, Sanders KM (1995) Impaired development of interstitial cells and intestinal electrical rhythmicity in steel mutants. Am J Physiol 269:C1577–C1585
Besmer P (1991) The kit ligand encoded at the murine Steel locus: a pleiotrophic growth and differentiation factor. Curr Opin Cell Biol 3:939–946
Maeda H, Yamagata A, Nishikawa S, Yoshinaga K, Kobayashi S, Nishi K, et al (1992) Requirement of c-kit for development of intestinal pacemaker system. Development 116:369–375
Wu J, Rothman TP, Gershon MD (1996) Development of interstitial cells of Cajal in the mouse gut: requirement for Kit ligand (KL). Neuroscience Abstract 22:31
Tsuzuki, T, Takayashi M, Asai N, Iwashita T, Matusyama M, Asai J (1995) Spatial and temporal expression of the ret proto-oncogene product in embryonic, infant, and adult rat tissues. Oncogene 10:191–198
Lecoin L, Gabella G, Le Douarin N (1996) Origin of the c-kit-positive interstitial cells in the avian bowel. Development 122:725–733
Miyazawa K, Williams DA, Gotoh A, Nishimaki J, Broxmeyer HE, Toyama K (1995) Membrane-bound steel factor induces more persistent tyrosine kinase activation and longer life span of c-kit gene-encoded protein than its soluble form. Blood 85:641–649
Wehrle-Haller B, Weston J (1995) Soluble and cell-bound forms of steel factor activity play distinct roles in melanocyte precursor dispersal and survival on the lateral neural crest migration pathway. Development 121:731–742
Angrist M, Bolk S, Thiel B, Puffenberger EG, Hofstra RM, Buys CH, et al (1995) Mutation analysis of the RET receptor tyrosine kinase in Hirschsprung disease. Hum Mol Genet 4:821–830
Edery P, Lyonnet S, Mulligan LM, Pelet A, Dow E, Abel L, et al (1994) Mutations of the RET proto-oncogene in Hirschsprung’s disease. Nature 367:378–380
Romeo G, Ronchetto P, Luo Y, Barone V, Seri M, Ceccherini I, et al (1994) Point mutations affect the tyrosine kinase domain of the RET proto-oncogene in Hirschsprung’s disease. Nature 367:377–378
Pasini B, Borrello M, Greco A, Bongarzone I, Luo Y, Mondellini P, et al (1995) Loss of function effect of RET mutations causing Hirschsprung disease. Nat Genet 10:35–40
Borrello MG, Smith DP, Pasini B, Bongarzone I, Greco A, Lorenzo MJ, et al (1995) RET activation by germline MEN2A and MEN2B mutations. Oncogene 11:2419–2427
Hofstra RM, Osinga J, Tan-Sindhunata G, Wu Y, Kamsteeg EJ, Stulp RP, et al (1996) A homozygous mutation in the endothelin-3 gene associated with a combined Waardenburg type 2 and Hirschsprung phenotype (Shah-Waardenburg syndrome). Nat Genet 12:445–447
Edery P, Attie T, Amiel J, Pelet A, Eng C, Hofstra RM, et al (1996) Mutation of the endothelin-3 gene in the Waardenburg-Hirschsprung disease (Shah-Waardenburg syndrome). Nat Genet 12:442–444
Fujimoto T, Hata J, Yokoyama S, Mitomi T (1989) A study of the extracellular matrix protein as the migration pathway of neural crest cells in the gut: analysis in human embryos with specific reference to the pathogenesis of Hirschsprung’s disease. J Pediatr Surg 24:550–556
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Gershon, M. (2008). Functional Anatomy of the Enteric Nervous System. In: Holschneider, A., Puri, P. (eds) Hirschsprung's Disease and Allied Disorders. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-33935-9_3
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