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.
Martini
R
R
Department of Neurology, Section of Developmental Neurobiology, University of Würzburg, Würzburg, Germany. neuk176@rzkli.uni-wuerzburg.de
D000818:Animals; D030801:Animals, Genetically Modified; D017630:Connexins / Q000235:genetics; D004268:DNA-Binding Proteins / Q000235:genetics; D004195:Disease Models, Animal; D051767:Early Growth Response Protein 2; D015316:Genetic Therapy; D015417:Hereditary Sensory and Motor Neuropathy / Q000235:genetics / Q000628:therapy; D051379:Mice; D008817:Mice, Mutant Strains; D009154:Mutation; D018993:Myelin P0 Protein / Q000235:genetics; D009185:Myelin Proteins / Q000235:genetics; D010523:Peripheral Nervous System Diseases / Q000235:genetics* / Q000628:therapy; D011129:Polyradiculoneuropathy / Q000235:genetics / Q000276:immunology / Q000628:therapy; D051381:Rats; D014157:Transcription Factors / Q000235:genetics; D000097002:Gap Junction beta-1 Protein
D017630:Connexins; D004268:DNA-Binding Proteins; D051767:Early Growth Response Protein 2; D018993:Myelin P0 Protein; D009185:Myelin Proteins; D014157:Transcription Factors; D000097002:Gap Junction beta-1 Protein; C497327:Egr2 protein, mouse; C497328:Egr2 protein, rat; C489545:Pmp22 protein, mouse; C489546:Pmp22 protein, rat