Summary
Intermitochondrial septate structures were found in the dystrophic axons of two cases of infantile neuroaxonal dystrophy. Septate structures were previously seen in some tumors (glioblastomas and Schwannomas) and several organs of vertebrates and invertebrates, but never in human central nervous system (CNS). The structures were studied by transmission and transmission tilt electron microscopy. A proposed model was constructed and X-rayed. Artist's depictions are shown and described. The intermitochondrial septate structures have a periodicity of 120 Å which puts them within the range of those structures previously reported. It was found that our structures are not true junctions, but a complex interdigitation of the outer membranes of the involved mitochondria.
Similar content being viewed by others
References
Bulger RE, Trump BF (1968) Occurrence of repeating septate subunits between apposed cellular membranes. Exp Cell Res 51:587–594
Conley FK, Herman MM (1973) Intracellular septate desmosome-like structures in a human acoustic Schwannoma in vitro. J Neurocytol 2:457–464
Lampert PW (1967) A comparative electron-microscopic study of reactive and degenerating, regenerating, and dystrophic axons. J Neuropath Exp Neurol 26:345–367
Jellinger K (1973) Neuroaxonal dystrophy: Its natural history and related disorders. In: Zimmerman HH (ed) Progress in neuropathology, vol 11, Grune and Stratton, New York, pp 129–180
Pearse AGE, Welsch U (1968) Ultrastructural characteristics of the thyroid C cells in the summer, autumn, and winter states of the hedgehog (Erinaceus europeaus L.), with some reference to other mammalian species. Z Zellforsch 92:596–609
Sandbank U, Lerman P, Geifman M (1970) Infantile neuroaxonal dystrophy: Cortical axonic and presynaptic changes. Acta Neuropathol (Berl) 16:342–352
Seitelberger F (1952) Eine unbekannte Form von infantiler Lipord-speicherkrankheit des Gehirns. In: Proceedings of the First International Congress of Neuropathology, vol 3, Rosenberg et Sellier, Turin, p 323
Tani E, Ametani T, Higashi N, Fujihara E (1971) Atypical cristae in mitochondria of human glioblastoma multiforme cells. J Ultrastruct Res 36:211–221
Wood RL (1959) Intercellular attachment in the epithelium of hydra as revealed by electron microscopy. J Biochem Biophys Cytol 6:343–351
Vernet G, Rué G, Gontcharoff M (1979) Etude par les techniques d'imprégnation au Lanthane et de cryofracture de jonctions septées de l'epithéium de la paroi du corps de lineus ruber (Hétéronémerte). J Ultrastruct Res 67:225–227
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Ramos, P.L., Wisniewski, K., Jervis, G.A. et al. Intermitochondrial septate structures in dystrophic axons. Acta Neuropathol 52, 105–109 (1980). https://doi.org/10.1007/BF00688007
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00688007