[Peripheral nerve abnormalities of mutant (PMA) mouse--myelinated fiber counts of sciatic, peroneal, sural and tibial nerves].
A mutant mouse characterized by peroneal muscular atrophy and congenital absence of the peroneal nerve has been described by Esaki et al. and investigated as a possible animal model of Charcot-Marie-Tooth disease or spinal muscular atrophy. However, the nature of the peripheral nerve abnormality of this mutant mouse has not been precisely defined yet. In this study, in addition to the qualitative evaluation of teased fiber and Epon-embedded preparations, the total transverse fascicular area and the total numbers of myelinated fibers per nerve in sciatic, peroneal (proximal and distal), sural (proximal and distal) and tibial (proximal and distal) nerves on both right and left sides were compared between six peroneal muscular atrophy (pma) mice with autosomal recessive gene manifesting mainly the peroneal muscular atrophy and their six control mice to understand and define the peripheral nerve abnormalities. The pma mice showed pes equinovarus bilaterally and their peroneal nerves were absent. No myelinated fibers showing axonal degeneration or segmental demyelination were found on teased fiber preparation. Onion bulb, demyelinated or remyelinated axons and myelin ovoids were not observed in Epon-embedded sections. The mean total transverse fascicular area and mean total number of myelinated fibers per nerve in the sciatic nerve in pma mice was significantly less (p less than 0.02) than that in control mice. On the other hand they were significantly greater (p less than 0.0001) in the sural nerve in pma mice than in control mice.(ABSTRACT TRUNCATED AT 250 WORDS)
A
A
Ohnishi
Y
Y
Kuroiwa
K
K
Esaki
D000818:Animals; D002452:Cell Count; D002607:Charcot-Marie-Tooth Disease / Q000473:pathology; D004195:Disease Models, Animal; D005260:Female; D005808:Genes, Recessive; D008297:Male; D051379:Mice; D008818:Mice, Neurologic Mutants / Q000033:anatomy & histology*; D009133:Muscular Atrophy / Q000151:congenital*; D009413:Nerve Fibers, Myelinated; D013118:Spinal Cord Diseases / Q000473:pathology; D013127:Spinal Nerves / Q000002:abnormalities*