Intermitochondrial septate structures in dystrophic axons.
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 A 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.
D001369:Axons / Q000648:ultrastructure*; D001927:Brain Diseases / Q000473:pathology*; D006801:Humans; D008566:Membranes / Q000648:ultrastructure; D008854:Microscopy, Electron; D008928:Mitochondria / Q000648:ultrastructure*