TY - JOUR
T1 - Heterozygous P0 knockout mice develop a peripheral neuropathy that resembles chronic inflammatory demyelinating polyneuropathy (CIDP)
AU - Shy, Michael E.
AU - Arroyo, Edgardo
AU - Sladky, John
AU - Menichella, Daniela
AU - Jiang, Huiyuan
AU - Xu, Wenbo
AU - Kamholz, John
AU - Scherer, Steven S.
N1 - Copyright:
Copyright 2018 Elsevier B.V., All rights reserved.
PY - 1997/7
Y1 - 1997/7
N2 - Demyelinating peripheral neuropathies are clinically divided into inherited and acquired types. Inherited demyelinating neuropathies are caused by mutations in genes expressed by myelinating Schwann cells, whereas acquired ones, including chronic inflammatory demyelinating polyneuropathy (CIDP), are probably caused by autoimmune mechanisms. We find that heterozygous P0 knockout (P0+/-) mice develop a neuropathy that resembles CIDP. By one year of age, P0 +/- mice develop severe, asymmetric slowing of motor nerves, with temporal dispersion or conduction block, which are features of acquired demyelinating neuropathies including CIDP. Moreover, morphological analysis of affected nerves reveals severe and selective demyelination of motor fibers, focal regions of demyelination, and inflammatory cells. These data suggest that immune-mediated mechanisms may contribute to the pathogenesis of the neuropathy in P0+/- mice.
AB - Demyelinating peripheral neuropathies are clinically divided into inherited and acquired types. Inherited demyelinating neuropathies are caused by mutations in genes expressed by myelinating Schwann cells, whereas acquired ones, including chronic inflammatory demyelinating polyneuropathy (CIDP), are probably caused by autoimmune mechanisms. We find that heterozygous P0 knockout (P0+/-) mice develop a neuropathy that resembles CIDP. By one year of age, P0 +/- mice develop severe, asymmetric slowing of motor nerves, with temporal dispersion or conduction block, which are features of acquired demyelinating neuropathies including CIDP. Moreover, morphological analysis of affected nerves reveals severe and selective demyelination of motor fibers, focal regions of demyelination, and inflammatory cells. These data suggest that immune-mediated mechanisms may contribute to the pathogenesis of the neuropathy in P0+/- mice.
KW - Axon-Schwann cell interactions
KW - CMT
KW - Cheroot Marie-Tooth disease
KW - Conduction block
KW - Demyelination
KW - My elin
KW - Schwann cells
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U2 - 10.1097/00005072-199756070-00008
DO - 10.1097/00005072-199756070-00008
M3 - Review article
C2 - 9210878
AN - SCOPUS:0030611992
VL - 56
SP - 811
EP - 821
JO - Journal of Neuropathology and Experimental Neurology
JF - Journal of Neuropathology and Experimental Neurology
SN - 0022-3069
IS - 7
ER -