Heterozygous P0 Knockout Mice Develop a Peripheral Neuropathy that Resembles Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

Michael Shy; Edgardo Arroyo; John Sladky; Daniela Menichella; Huiyuan Jiang; Wenbo Xu; John Kamholz; Steven Scherer

doi:10.1097/00005072-199756070-00008

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Heterozygous P0 Knockout Mice Develop a Peripheral Neuropathy that Resembles Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

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Heterozygous P0 Knockout Mice Develop a Peripheral Neuropathy that Resembles Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

Michael Shy, Edgardo Arroyo, John Sladky, Daniela Menichella, Huiyuan Jiang, Wenbo Xu, John Kamholz and Steven Scherer

Journal of neuropathology and experimental neurology, Vol.56(7), pp.811-821

07/1997

DOI: 10.1097/00005072-199756070-00008

PMID: 9210878

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Abstract

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.

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Title: Subtitle: Heterozygous P0 Knockout Mice Develop a Peripheral Neuropathy that Resembles Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
Creators: Michael Shy - From the Department of Neurology & Center for Molecular Medicine and Genetics, Wayne State University, Detroit, Michigan (MES, DM, HJ, WX, JK), the Department of Neurology, the University of Pennsylvania Medical Center, Philadelphia, Pennsylvania (EA, SSS), the Division of Pediatric Neurology, Emory University School of Medicine, Atlanta, Georgia (JS), and the Institute of Neurology, Dino Ferrari Center IRCCS, University of Milan, Italy IRCCS (DM)
Edgardo Arroyo
John Sladky
Daniela Menichella
Huiyuan Jiang
Wenbo Xu
John Kamholz
Steven Scherer
Resource Type: Journal article
Publication Details: Journal of neuropathology and experimental neurology, Vol.56(7), pp.811-821
DOI: 10.1097/00005072-199756070-00008
PMID: 9210878
NLM abbreviation: J Neuropathol Exp Neurol
ISSN: 0022-3069
eISSN: 1554-6578
Publisher: American Association of Neuropathologists, Inc
Language: English
Date published: 07/1997
Academic Unit: Neurology; Molecular Physiology and Biophysics; Psychiatry; Stead Family Department of Pediatrics; Iowa Neuroscience Institute
Record Identifier: 9984020864402771

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