Attenuated demyelination in the absence of the macrophage-restricted adhesion molecule sialoadhesin (Siglec-1) in mice heterozygously deficient in P0. Mol Cell Neurosci 2006 Apr;31(4):685-91
Date
02/07/2006Pubmed ID
16458537DOI
10.1016/j.mcn.2005.12.007Scopus ID
2-s2.0-33645349039 (requires institutional sign-in at Scopus site) 37 CitationsAbstract
Mouse mutants heterozygously deficient for the myelin component P0 mimic some forms of inherited neuropathies in humans. We have previously shown that both T lymphocytes and macrophages contribute to the demyelinating neuropathy. Both cell types appear to influence each other mutually, i.e., impaired T lymphocyte development in RAG-1-deficient P0 mutants leads to decreased macrophage numbers and retarded macrophage activation causes reduced T lymphocyte numbers in the peripheral nerves of P0(+/-) mice. In the present study, we investigated the possible role of the macrophage-restricted sialic acid-binding Ig-like lectin sialoadhesin (Sn, Siglec-1) in the pathogenesis of inherited demyelination in P0(+/-) mice. We found that most peripheral nerve macrophages express Sn in the mutants. Myelin mutants devoid of Sn show reduced numbers of CD8+ T lymphocytes and macrophages in peripheral nerves and less severe demyelination, resulting in improved nerve conduction properties. Our findings are potentially important in the development of future treatment strategies for inherited demyelinating neuropathies.
Author List
Kobsar I, Oetke C, Kroner A, Wessig C, Crocker P, Martini RAuthor
Antje Kroner-Milsch MD, PhD Associate Professor in the Neurosurgery department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AnimalsCD8-Positive T-Lymphocytes
Charcot-Marie-Tooth Disease
Demyelinating Diseases
Disease Models, Animal
Electrophysiology
Humans
Macrophages
Membrane Glycoproteins
Mice
Mice, Knockout
Myelin P0 Protein
Myelin Sheath
Peripheral Nerves
Phenotype
Receptors, Immunologic
Sialic Acid Binding Ig-like Lectin 1
