Schwann Cells Research from Zurich

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(Understanding Schwann cell neurone interactions; the key to Charcot

Marie Tooth Disease?)

1: J Anat 2002 Apr;200(4):357-66

Understanding Schwann cell-neurone interactions: the key to

Charcot-Marie-Tooth disease?

Maier M, Berger P, Suter U.

Institute of Cell Biology, Department of Biology, Swiss Federal

Institute of Technology, ETH-Honggerberg, Zurich.

ABSTRACT:

Charcot-Marie-Tooth disease (CMT) comprises a heterogeneous group of

disorders. The most frequent subtype is caused by increased PMP22 gene

dosage or missense point mutations affecting the PMP22 gene (CMT type

1A; CMT1A). Animal models in rat and mouse with the corresponding PMP22

alterations are available and mimic many aspects of the human diseases.

Complementary data from patients, point towards altered Schwann

cell-neurone interactions as a major underlying mechanism of CMT1A and

related hereditary neuropathies. This is evident from the finding that

mutated proteins affecting either Schwann cells or neurones have a

profound influence on their partner cells. Recently, a number of novel

genes causing various forms of CMT have been identified which are

expressed either mainly by Schwann cells and/or by the

accompanying neurones. These genes can be viewed, in analogy to classic

experiments routinely performed in lower vertebrates, as the result of a

'functional screen' revealing crucial players in the interactions

between Schwann cells and neurones. Studying how Schwann cell and

axon-encoded proteins are functionally interconnected will be an

exciting task for the future. It will not only yield insights into the

molecular and cellular basis of neuropathies but also provide crucial

information about the interplay between Schwann cells and neurones in

general.