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Article

A rat model of Charcot-Marie-Tooth disease 1A recapitulates disease variability and supplies biomarkers of axonal loss in patients.

Brain : a journal of neurology - 1 Jan 2012

Fledrich Robert, Schlotter-Weigel Beate, Schnizer Tuuli J, Wichert Sven P, Stassart Ruth M, Meyer zu Hörste Gerd, Klink Axel, Weiss Bernhard G, Haag Uwe, Walter Maggie C, Rautenstrauss Bernd, Paulus Walter, Rossner Moritz J, Sereda Michael W

Abstract excerpt

Charcot-Marie-Tooth disease is the most common inherited neuropathy and a duplication of the peripheral myelin protein 22 gene causes the most frequent subform Charcot-Marie-Tooth 1A. Patients develop a slowly progressive dysmyelinating and demyelinating peripheral neuropathy and distally pronounced muscle atrophy. The amount of axonal loss determines disease severity. Although patients share an identical...

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PMID: 22189569
DOI: 10.1093/brain/awr322

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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