title: A novel pathogenesis of megacolon in Ncx/Hox11L.1 deficient mice.
abstract: The Ncx/Hox11L.1 gene, a member of the Hox11 homeobox gene family, is mainly expressed in neural crest-derived tissues. To elucidate the role of Ncx/Hox11L.1, the gene has been inactivated in embryonic stem cells by homologous recombination. The homozygous mutant mice were viable. These mice developed megacolon with enteric ganglia by age 3-5 wk. Histochemical analysis of the ganglia revealed that the enteric neurons hyperinnervated in the narrow segment of megacolon. Some of these neuronal cells degenerated and neuronal cell death occurred in later stages. We propose that Ncx/Hox11L.1 is required for maintenance of proper functions of the enteric nervous system. These mutant mice can be used to elucidate a novel pathogenesis for human neuronal intestinal dysplasia.
authors.affiliation: Division of Developmental Genetics, Chiba University School of Medicine, Chiba 260, Japan. hatano@med.m.chiba-u.ac.jp
authors.forename: M
authors.initials: M
authors.lastname: Hatano
authors.forename: T
authors.initials: T
authors.lastname: Aoki
authors.forename: M
authors.initials: M
authors.lastname: Dezawa
authors.forename: S
authors.initials: S
authors.lastname: Yusa
authors.forename: Y
authors.initials: Y
authors.lastname: Iitsuka
authors.forename: H
authors.initials: H
authors.lastname: Koseki
authors.forename: M
authors.initials: M
authors.lastname: Taniguchi
authors.forename: T
authors.initials: T
authors.lastname: Tokuhisa
mesh_terms: D000818:Animals; D003106:Colon / Q000737:chemistry / Q000294:innervation* / Q000473:pathology / Q000648:ultrastructure; D017615:Enteric Nervous System / Q000737:chemistry / Q000648:ultrastructure; D005724:Ganglia / Q000737:chemistry / Q000473:pathology / Q000648:ultrastructure; D018398:Homeodomain Proteins / Q000378:metabolism / Q000502:physiology*; D007150:Immunohistochemistry; D008531:Megacolon / Q000209:etiology* / Q000235:genetics; D051379:Mice; D018345:Mice, Knockout; D009252:NADPH Dehydrogenase / Q000032:analysis; D009478:Neuropeptide Y / Q000032:analysis; D015513:Oncogene Proteins / Q000172:deficiency / Q000378:metabolism / Q000502:physiology*; D013373:Substance P / Q000032:analysis
chemical_list: D018398:Homeodomain Proteins; D009478:Neuropeptide Y; D015513:Oncogene Proteins; C487378:Tlx1 protein, mouse; D013373:Substance P; D009252:NADPH Dehydrogenase