title: Substance P immunoreactivity in Rett syndrome.
abstract: Severe autonomic dysfunction occurs in Rett syndrome (RS). Substance P, a tachykinin peptide that localizes to several brain regions, including the autonomic nervous system, is reduced in the cerebrospinal fluid of patients with RS. The anatomic localization and intensity of substance P immunoreactivity and glial fibrillary acidic protein-positive astrocytes in the brains of 14 patients with RS were compared with those in the brains of 10 age-matched normal patients. Substance P immunoreactivity expression was significantly decreased in RS tissue compared with control tissue in the following regions: dorsal horns, intermediolateral column of the spinal cord, spinal trigeminal tract, solitary tract and nucleus, parvocellular and pontine reticular nuclei, and locus ceruleus. A less significant decrease of substance P immunoreactivity occurred in the substantia nigra, central gray of the midbrain, frontal cortex, caudate, putamen, globus pallidus, and thalamus. Antiglial fibrillary acidic protein-positive astrocytes were increased in the areas in which substance P immunoreactivity was decreased and in other brain regions. Because many of the brain regions with the greatest decrease in substance P immunoreactivity are involved in the control of the autonomic nervous system, especially the solitary tracts and reticular formation, reduced substance P may contribute to the autonomic dysfunction in RS.
authors.affiliation: Rett Center, Houston, TX, USA.
authors.forename: K
authors.initials: K
authors.lastname: Deguchi
authors.forename: B A
authors.initials: BA
authors.lastname: Antalffy
authors.forename: L J
authors.initials: LJ
authors.lastname: Twohill
authors.forename: S
authors.initials: S
authors.lastname: Chakraborty
authors.forename: D G
authors.initials: DG
authors.lastname: Glaze
authors.forename: D D
authors.initials: DD
authors.lastname: Armstrong
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000906:Antibodies; D001253:Astrocytes / Q000737:chemistry / Q000473:pathology; D001479:Basal Ganglia / Q000737:chemistry / Q000473:pathology; D001921:Brain / Q000473:pathology*; D001923:Brain Chemistry; D001933:Brain Stem / Q000737:chemistry / Q000473:pathology; D002648:Child; D005260:Female; D005625:Frontal Lobe / Q000737:chemistry / Q000473:pathology; D005904:Glial Fibrillary Acidic Protein / Q000032:analysis / Q000276:immunology; D006624:Hippocampus / Q000737:chemistry / Q000473:pathology; D006801:Humans; D008636:Mesencephalon / Q000737:chemistry / Q000473:pathology; D009474:Neurons / Q000737:chemistry / Q000473:pathology; D015518:Rett Syndrome / Q000473:pathology*; D013373:Substance P / Q000032:analysis* / Q000276:immunology; D013788:Thalamus / Q000737:chemistry / Q000473:pathology
chemical_list: D000906:Antibodies; D005904:Glial Fibrillary Acidic Protein; D013373:Substance P