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Functional recovery of regenerating motor axons is delayed in mice heterozygously deficient for the myelin protein P(0) gene

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Mice with a heterozygous knock-out of the myelin protein P0 gene (P0+/-) develop a neuropathy similar to human Charcot-Marie-Tooth disease. They are indistinguishable from wild-types (WT) at birth and develop a slowly progressing demyelinating neuropathy. The aim of this study was to investigate whether the regeneration capacity of early symptomatic P0+/- is impaired as compared to age matched WT. Right sciatic nerves were lesioned at the thigh in 7-8 months old mice. Tibial motor axons at ankle were investigated by conventional motor conduction studies and axon excitability studies using threshold tracking. To evaluate regeneration we monitored the recovery of motor function after crush, and then compared the fiber distribution by histology. The overall motor performance was investigated using Rotor-Rod. P0+/- had reduced compound motor action potential amplitudes and thinner myelinated axons with only a borderline impairment in conduction and Rotor-Rod. Plantar muscle reinnervation occurred within 21 days in all mice. Shortly after reinnervation the conduction of P0+/- regenerated axons was markedly slower than WT, however, this difference decayed with time. Nevertheless, after 1 month, regenerated P0+/- axons had longer strength-duration time constant, larger threshold changes during hyperpolarizing electrotonus and longer relative refractory period. Their performance at Rotor-Rod remained also markedly impaired. In contrast, the number and diameter distribution of regenerating myelinated fibers became similar to regenerated WT. Our data suggest that in the presence of heterozygously P0 deficient Schwann cells, regenerating motor axons retain their ability to reinnervate their targets and remyelinate, though their functional recovery is delayed.
Original languageEnglish
JournalNeurochemical Research
Volume38
Issue number6
Pages (from-to)1266-77
Number of pages12
ISSN0364-3190
DOIs
Publication statusPublished - Jun 2013

    Research areas

  • Action Potentials, Animals, Axons, Behavior, Animal, Charcot-Marie-Tooth Disease, Disease Models, Animal, Heterozygote, Mice, Motor Neurons, Myelin P0 Protein, Nerve Regeneration, Neural Conduction, Postural Balance, Psychomotor Performance, Rotarod Performance Test, Tibial Nerve

ID: 42521489