Cell adhesion molecule N-CAM is expressed by denervated myofibres in Werdnig-Hoffman and Kugelberg-Welander type spinal muscular atrophies

17Citations
Citations of this article
6Readers
Mendeley users who have this article in their library.

Abstract

Immunocytochemical analysis utilising antibody to neural cell adhesion molecule (N-CAM) was carried out on skeletal muscle biopsies from patients with childhood spinal muscular atrophy. Children with both Werdnig-Hoffmann and Kugelberg-Welander disease showed positive N-CAM reactivity. There were however differences in the N-CAM expression profiles in these two sets of patients. All myofibres were positive for N-CAM in the Werdnig-Hoffmann patients. This included both the normal sized fibres and the atrophic fibres. In contrast only the atrophic fibres were positive in the Kugelberg-Welander patients. No reactivity was found associated with the large hypertrophic fibres. It is likely that in the Werdnig-Hoffmann patients the positive N-CAM reactivity reflects unstable innervation of myofibres that had been previously innervated. A similar mechanism may operate in the Kugelberg-Welander patients, but the innervation of the hypertrophic fibres is more stable as they are able to repress N-CAM expression. These results contrast with a lack of N-CAM expression found previously on muscle biopsies from adults with denervation disease.

Cite

CITATION STYLE

APA

Walsh, F. S., Moore, S. E., & Lake, B. D. (1987). Cell adhesion molecule N-CAM is expressed by denervated myofibres in Werdnig-Hoffman and Kugelberg-Welander type spinal muscular atrophies. Journal of Neurology Neurosurgery and Psychiatry, 50(4), 439–442. https://doi.org/10.1136/jnnp.50.4.439

Register to see more suggestions

Mendeley helps you to discover research relevant for your work.

Already have an account?

Save time finding and organizing research with Mendeley

Sign up for free