Immune deficiency in mouse models for inherited peripheral neuropathies leads to improved myelin maintenance.
J Neurosci
; 20(2): 729-35, 2000 Jan 15.
Article
em En
| MEDLINE
| ID: mdl-10632602
ABSTRACT
The adhesive cell surface molecule P(0) is the most abundant glycoprotein in peripheral nerve myelin and fulfills pivotal functions during myelin formation and maintenance. Mutations in the corresponding gene cause hereditary demyelinating neuropathies. In mice heterozygously deficient in P(0) (P(0)(+/-) mice), an established animal model for a subtype of hereditary neuropathies, T-lymphocytes are present in the demyelinating nerves. To monitor the possible involvement of the immune system in myelin pathology, we cross-bred P(0)(+/-) mice with null mutants for the recombination activating gene 1 (RAG-1) or with mice deficient in the T-cell receptor alpha-subunit. We found that in P(0)(+/-) mice myelin degeneration and impairment of nerve conduction properties is less severe when the immune system is deficient. Moreover, isolated T-lymphocytes from P(0)(+/-) mice show enhanced reactivity to myelin components of the peripheral nerve, such as P(0), P(2), and myelin basic protein. We hypothesize that autoreactive immune cells can significantly foster the demyelinating phenotype of mice with a primarily genetically based peripheral neuropathy.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Linfócitos T
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Doenças Desmielinizantes
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Receptores de Antígenos de Linfócitos T alfa-beta
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Genes RAG-1
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Doenças do Sistema Nervoso Periférico
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Proteínas de Homeodomínio
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Proteína P0 da Mielina
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Síndromes de Imunodeficiência
Limite:
Animals
Idioma:
En
Revista:
J Neurosci
Ano de publicação:
2000
Tipo de documento:
Article
País de afiliação:
Alemanha