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The role of the immune system in hereditary neuropathies

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Abstract from Nervenarzt. 2004 Dec 4

The role of the immune system in hereditary neuropathies.

[Article in German]

Maurer M, Toyka KV, i R.

Neurologische Universitatsklinik Wurzburg.

Hereditary neuropathies, e.g., Charcot-Marie-Tooth (CMT) disease, are

inherited diseases of the peripheral nervous system causing chronic

progressive motor and sensory dysfunction. Most neuropathies are due

to mutations in myelin genes such as PMP22, P0, and the gap junction

protein Cx32. Myelin mutant mice are regarded as suitable animal

models for several forms of hereditary neuropathies and are important

neurobiological tools for the evaluation of pathogenetic and

therapeutic concepts in hereditary neuropathies.

Using these animal models we could recently show that the immune

system is involved in the pathogenesis of hereditary neuropathies.

Due to the phenotypic similarities we also consider the immune system

important for human inherited neuropathies, in particular since

several case reports demonstrate a beneficial effect of immune

therapies in patients with hereditary neuropathies. In this review we

compare findings from animal models and human disease to elucidate

the role of the immune system in hereditary neuropathies.

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