Major histocompatibility complex class II expression and macrophage responses in genetically proven Charcot-Marie-Tooth type 1 and hereditary neuropathy with liability to pressure palsies

Stoll, Guido; Gabreëls-Festen, A.A.W.M.; Jander, Sebastian; Müller, Hans; Hanemann, C Oliver

doi:10.1002/(sici)1097-4598(199811)21:11<1419::aid-mus9>3.0.co;2-c

Cited by 15 publications

(7 citation statements)

References 37 publications

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“…Similar to our animal model, an increased number of endoneurial macrophages has also been reported in nerve biopsies of patients with inherited peripheral neuropathies (Sommer and Schröder, 1995;Schmidt et al, 1996;Stoll et al, 1998). In many forms of CMT the active phase of demyelination occurs during childhood (Gabreëls-Festen et al, 1995;Garcia et al, 1998;Thomas, 1999), and nerve biopsies were usually not performed.…”

Section: The Role Of Macrophages In Human Inherited Neuropathiessupporting

confidence: 82%

The Role of Macrophages in Demyelinating Peripheral Nervous System of Mice Heterozygously Deficient in P0

Carenini

Mäurer

Werner

et al. 2001

The Journal of Cell Biology

115

116

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Mice heterozygously deficient in the p0 gene (P0+/−) are animal models for some forms of inherited neuropathies. They display a progressive demyelinating phenotype in motor nerves, accompanied by mild infiltration of lymphocytes and increase in macrophages. We have shown previously that the T lymphocytes are instrumental in the demyelination process. This study addresses the functional role of the macrophage in this monogenic myelin disorder.In motor nerves of P0+/− mice, the number of macrophages in demyelinated peripheral nerves was increased by a factor of five when compared with motor nerves of wild-type mice. Immunoelectron microscopy, using a specific marker for mouse macrophages, displayed macrophages not only in the endoneurium of the myelin mutants, but also within endoneurial tubes, suggesting an active role in demyelination. To elucidate the roles of the macrophages, we crossbred the myelin mutants with a spontaneous mouse mutant deficient in macrophage colony-stimulating factor (M-CSF), hence displaying impaired macrophage activation. In the P0-deficient double mutants also deficient in M-CSF, the numbers of macrophages were not elevated in the demyelinating motor nerves and demyelination was less severe. These findings demonstrate an active role of macrophages during pathogenesis of inherited demyelination with putative impact on future treatment strategies.

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Section: The Role Of Macrophages In Human Inherited Neuropathiessupporting

confidence: 82%

The Role of Macrophages in Demyelinating Peripheral Nervous System of Mice Heterozygously Deficient in P0

Carenini

Mäurer

Werner

et al. 2001

The Journal of Cell Biology

115

116

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“…Furthermore, hereditary neuropathies may have a late onset, and T-cells and macrophages may be found in their sural nerve biopsies. 7,16,17 The notion that clustering of macrophages around endoneurial vessels could be an indicator for an underlying CIDP is supported by experimental findings. In rabbits a chronic demyelinating neuropathy (experimental autoimmune neuritis [EAN]) can be induced by repeated sensitization against galactocerebroside (GalC).…”

Section: Figure 2 Representative Examples Of Macrophages (Cd 68; a mentioning

confidence: 83%

Macrophage clustering as a diagnostic marker in sural nerve biopsies of patients with CIDP

Sommer¹,

Koch²,

Lammens³

et al. 2005

Neurology

112

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“…Apart from macrophage‐associated demyelination, which is characteristic of Guillain–Barré syndrome and related disorders, an increased number of endoneurial macrophages has also been reported in nerve biopsies from patients with inherited neuropathy 17. It is conceivable that circulating monocytes enter the peripheral nerve to clear the myelin debris that results from genetically mediated disintegration of myelin.…”

Section: Discussionmentioning

confidence: 99%

Inflammatory demyelination in a patient with CMT1A

Vital¹,

Vital²,

Lagueny³

et al. 2003

Muscle and Nerve

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We report a case of Charcot-Marie-Tooth disease (CMT), with identified PMP22 gene duplication (CMT type 1A), and with evidence of an inflammatory demyelinating process superimposed on the course of the chronic genetic disease. Macrophage-associated demyelination was observed on the peripheral nerve biopsy. This observation supports some experimental data from the literature and shows that there may be a genetic susceptibility to inflammatory demyelinating processes in certain CMT kindreds.

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Major histocompatibility complex class II expression and macrophage responses in genetically proven Charcot-Marie-Tooth type 1 and hereditary neuropathy with liability to pressure palsies

Cited by 15 publications

References 37 publications

The Role of Macrophages in Demyelinating Peripheral Nervous System of Mice Heterozygously Deficient in P0

The Role of Macrophages in Demyelinating Peripheral Nervous System of Mice Heterozygously Deficient in P0

Macrophage clustering as a diagnostic marker in sural nerve biopsies of patients with CIDP

Inflammatory demyelination in a patient with CMT1A

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