Animal models for inherited peripheral neuropathies: chances to find treatment strategies?

Mutations in four identified genes (peripheral myelin protein 22, P(0), connexin 32, and the early growth response 2 zinc finger protein) are the cause for several forms of inherited peripheral neuropathies that are still incurable disorders. Some forms of these disorders are well mimicked by engineered or spontaneous rodent mutants that might be instrumental for developing treatment strategies. This review focusses on common pathways of pathogenesis of the disorders and emphasizes strategies that might be suitable to ameliorate disease expression.