title: Cortical and brainstem neurons containing calcium-binding proteins in a murine model of Duchenne's muscular dystrophy: selective changes in the sensorimotor cortex.
abstract: In the muscular dystrophic (mdx) mouse, which is characterized by deficient dystrophin expression and provides a model of Duchenne's muscular dystrophy, we previously demonstrated marked central nervous system alterations and in particular a quantitative reduction of corticospinal and rubrospinal neurons and pathologic changes of these cells. Prompted by these findings and in view of the relations between calcium ions and dystrophin, we analyzed with immunohistochemistry the neurons containing the calcium-binding proteins parvalbumin, calbindin D28k, and calretinin in cortical areas and brainstem nuclei of mdx mice. In the sensorimotor cortex, parvalbumin-positive and calbindin-positive neurons, which represent a subset of cortical interneurons, were significantly more numerous in mdx mice than in wild-type ones. In addition, the laminar distribution of parvalbumin-positive neurons in the motor and somatosensory cortical areas of mdx mice was altered with respect to wild-type animals. No alterations in the number and distribution were found in the parvalbumin- or calbindin-expressing cell populations of the visual and anterior cingulate cortices of mdx mice. The pattern of calretinin immunoreactivity was normal in all investigated cortical areas. The cell populations containing either calcium-binding protein were similar in brainstem nuclei of mdx and wild-type mice. The present findings demonstrated selective changes of subsets of interneurons in the motor and somatosensory cortical areas of mdx mice. Therefore, the data showed that, in the cortices of these mutant animals, the previously demonstrated pathologic changes of corticospinal cell populations are accompanied by marked alterations in the local circuitry.
authors.affiliation: Department of Neurological and Psychiatric Sciences, University of Florence, Florence, Italy, I-50134.
authors.forename: Donatella
authors.initials: D
authors.lastname: Carretta
authors.forename: Marialaura
authors.initials: M
authors.lastname: Santarelli
authors.forename: Duccio
authors.initials: D
authors.lastname: Vanni
authors.forename: Sonia
authors.initials: S
authors.lastname: Ciabatti
authors.forename: Alessandro
authors.initials: A
authors.lastname: Sbriccoli
authors.forename: Francesco
authors.initials: F
authors.lastname: Pinto
authors.forename: Diego
authors.initials: D
authors.lastname: Minciacchi
mesh_terms: D000818:Animals; D001933:Brain Stem / Q000378:metabolism*; D064092:Calbindin 1; D064032:Calbindin 2; D064026:Calbindins; D002135:Calcium-Binding Proteins / Q000378:metabolism*; D002540:Cerebral Cortex / Q000378:metabolism*; D004195:Disease Models, Animal; D006179:Gyrus Cinguli / Q000378:metabolism; D007150:Immunohistochemistry; D007395:Interneurons / Q000378:metabolism*; D051379:Mice; D008810:Mice, Inbred C57BL; D008818:Mice, Neurologic Mutants; D009044:Motor Cortex / Q000378:metabolism; D020388:Muscular Dystrophy, Duchenne / Q000378:metabolism*; D010320:Parvalbumins / Q000378:metabolism; D064030:S100 Calcium Binding Protein G / Q000378:metabolism; D013003:Somatosensory Cortex / Q000378:metabolism*; D014793:Visual Cortex / Q000378:metabolism
chemical_list: C576499:Calb1 protein, mouse; C576511:Calb2 protein, mouse; D064092:Calbindin 1; D064032:Calbindin 2; D064026:Calbindins; D002135:Calcium-Binding Proteins; D010320:Parvalbumins; D064030:S100 Calcium Binding Protein G