title: Lack of a docking mechanism for neurotransmitter release in the aganglionic segment of bowel in patients with Hirschsprung's disease.
abstract: We examined immunohistochemically the expression and localisation of synapse-associated proteins, syntaxin (SNT) and synaptotagmin (STG) in the entire resected specimens of colon obtained at the time of pull-through operation from 15 patients with Hirschsprung's disease (HD) and 6 age-matched controls. Both antibodies showed a similar pattern of staining. In the normal colon and ganglionic colon from HD, there was strong reactivity in the submucous and myenteric plexuses in addition to staining of nerve fibres in the smooth-muscle layers. In the aganglionic colon, there was an absence or marked decrease in SNT and STG-positive nerve fibres in the smooth-muscle layers and in hypertrophic nerve trunks. Our data indicate that important proteins necessary for the docking of synaptic vesicles at the presynaptic plasma membrane are lost in fibres innervating the smooth muscle of HD and suggest that abnormal neurotransmission may have a role in the maintenance of muscle spasticity.
authors.affiliation: Children's Research Centre, Our Lady's Hospital for Sick Children, Crumlin, Dublin 12, Ireland.
authors.forename: Y
authors.initials: Y
authors.lastname: Kakita
authors.forename: K
authors.initials: K
authors.lastname: Ohshiro
authors.forename: P
authors.initials: P
authors.lastname: Puri
authors.forename: H
authors.initials: H
authors.lastname: Kobayashi
authors.forename: D S
authors.initials: DS
authors.lastname: O'Briain
mesh_terms: D002135:Calcium-Binding Proteins; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D003106:Colon / Q000294:innervation* / Q000378:metabolism; D006627:Hirschsprung Disease / Q000378:metabolism* / Q000601:surgery; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D007231:Infant, Newborn; D008562:Membrane Glycoproteins / Q000378:metabolism*; D008565:Membrane Proteins / Q000378:metabolism*; D009419:Nerve Tissue Proteins / Q000378:metabolism*; D050765:Qa-SNARE Proteins; D050857:Synaptotagmins
chemical_list: D002135:Calcium-Binding Proteins; D008562:Membrane Glycoproteins; D008565:Membrane Proteins; D009419:Nerve Tissue Proteins; D050765:Qa-SNARE Proteins; D050857:Synaptotagmins