The organisation of spinal projecting brainstem neurons in an animal model of muscular dystrophy: A retrograde tracing study on mdx mutant mice

• Published in: Brain research Vol. 895; no. 1; pp. 213 - 222
• Main Authors: Carretta, Donatella, Santarelli, Marialaura, Vanni, Duccio, Carrai, Riccardo, Sbriccoli, Alessandro, Pinto, Francesco, Minciacchi, Diego
• Format: Journal Article
• Language: English
• Published: London Elsevier B.V 23.03.2001; Amsterdam Elsevier; New York, NY
• Subjects: Animals; Axonal Transport - drug effects; Axonal Transport - physiology; Biological and medical sciences; Brain Stem - pathology; Brain Stem - physiopathology; Cell Count; Descending motor projection; Disease Models, Animal; Diseases of striated muscles. Neuromuscular diseases; Duchenne muscular dystrophy; Efferent Pathways - pathology; Efferent Pathways - physiopathology; Medical sciences; Mice; Mice, Inbred C57BL; Mice, Inbred mdx; Motor system; Muscular Dystrophy, Animal - pathology; Muscular Dystrophy, Animal - physiopathology; Muscular Dystrophy, Duchenne - pathology; Muscular Dystrophy, Duchenne - physiopathology; Neurology; Neurons - pathology; Raphe Nuclei - pathology; Raphe Nuclei - physiopathology; Red nucleus; Red Nucleus - pathology; Red Nucleus - physiopathology; Reticular Formation - pathology; Reticular Formation - physiopathology; Spinal Cord - pathology; Spinal Cord - physiopathology; Vestibular Nuclei - pathology; Vestibular Nuclei - physiopathology; Wheat Germ Agglutinin-Horseradish Peroxidase Conjugate - pharmacokinetics; Wheat germ agglutinin–horseradish peroxidase; Disorders of the nervous system; Descending motor projection; Red nucleus; Duchenne muscular dystrophy; Wheat germ agglutinin–horseradish peroxidase; Motor system; Animal model; Neuromuscular diseases; Nervous system diseases; Spinal cord; Brain stem; Rodentia; Central nervous system; Projection; Genetic disease; Vertebrata; Mammalia; Mouse; Mutation; Brain (vertebrata)
• ISSN: 0006-8993; 1872-6240
• DOI: 10.1016/S0006-8993(01)02108-4

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.