The organisation of spinal projecting brainstem neurons in an animal model of muscular dystrophy. A retrograde tracing study on mdx mutant mice.
Previous studies we performed on the mdx mouse demonstrated marked central nervous system alterations in this model of human Duchenne muscular dystrophy, such as reduction in number and pathological changes of cortico-spinal neurons. Prompted by these findings we extended the survey of the mdx brain to the major brainstem-descending pathways: the rubro-, vestibulo-, reticulo-, and raphe-spinal projections. Horseradish peroxidase microinjections were performed in the cervical spinal cord of mdx and control mice. The rubro-spinal neurons were found to be significantly reduced in mutants compared to controls. The vestibulo-spinal, reticulo-spinal, and raphe-spinal cell populations, though less numerous in mdx than in control mice, were instead substantially spared. Our data further unveil the selective nature of mdx brain damage indicating a marked and selective involvement of the highest centers for motor control.
Department of Neurological and Psychiatric Sciences, University of Florence, Viale Morgagni 85, I-50134, Florence, Italy.
D
D
Carretta
M
M
Santarelli
D
D
Vanni
R
R
Carrai
A
A
Sbriccoli
F
F
Pinto
D
D
Minciacchi
D000818:Animals; D001370:Axonal Transport / Q000187:drug effects / Q000502:physiology; D001933:Brain Stem / Q000473:pathology* / Q000503:physiopathology; D002452:Cell Count; D004195:Disease Models, Animal; D004525:Efferent Pathways / Q000473:pathology* / Q000503:physiopathology; D051379:Mice; D008810:Mice, Inbred C57BL; D018101:Mice, Inbred mdx; D009137:Muscular Dystrophy, Animal / Q000473:pathology* / Q000503:physiopathology; D020388:Muscular Dystrophy, Duchenne / Q000473:pathology* / Q000503:physiopathology; D009474:Neurons / Q000473:pathology*; D011903:Raphe Nuclei / Q000473:pathology / Q000503:physiopathology; D012012:Red Nucleus / Q000473:pathology / Q000503:physiopathology; D012154:Reticular Formation / Q000473:pathology / Q000503:physiopathology; D013116:Spinal Cord / Q000473:pathology* / Q000503:physiopathology; D014726:Vestibular Nuclei / Q000473:pathology / Q000503:physiopathology; D018998:Wheat Germ Agglutinin-Horseradish Peroxidase Conjugate / Q000493:pharmacokinetics
D018998:Wheat Germ Agglutinin-Horseradish Peroxidase Conjugate