P0 protein is a target antigen in chronic inflammatory demyelinating polyradiculoneuropathy

Yan, Wei; Archelos, Juan J.; Hartung, Hans‐Peter; Pollard, John D.

doi:10.1002/ana.1129

Cited by 151 publications

(95 citation statements)

References 32 publications

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“…In the study of Yan et al western immunoblot technique identified antibodies to P0 in six of 21 CIDP patients who were responsive to plasma exchange and only in one of 15 controls. 15 Allen and colleagues also found antibodies to P0-like bands by immunoblot in eight of 32 patients with CIDP and none in 30 normal controls. 16 However, Sanvito et al did not find a significant difference in the proportion of subjects with IgG anti-P0 peptides in patients with CIDP compared with controls, although individual subjects with CIDP had high titers.…”

Section: Dovepressmentioning

confidence: 98%

“…22 Furthermore, the antibodies to P0 glycoprotein in Yan and colleagues' study were mainly IgG 1, a subclass that implies T-cell activation. 15 It is likely that both B and T-cell mechanisms are involved. More research is needed to establish the target of the T-cell response and whether other cell populations, like NK and T-cells are relevant to the pathogenesis of CIDP.…”

Section: Dovepressmentioning

confidence: 99%

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Overview of the pathogenesis and treatment of chronic inflammatory demyelinating polyneuropathy with intravenous immunoglobulins

Mahdi-Rogers

Rajabally²

2010

BTT

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Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired heterogeneous disorder of immune origin affecting the peripheral nerves, causing motor weakness and sensory symptoms and signs. The precise pathophysiology of CIDP remains uncertain although B and T cell mechanisms are believed to be implicated. Intravenous immunoglobulins (IVIg) have been shown in a number of trials to be an effective treatment for CIDP. IVIg is thought to exert its immunomodulatory effects by affecting several components of the immune system including B-cells, T-cells, macrophages and complement. This article provides an overview of the pathogenesis of CIDP and of its treatment with IVIg.

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Section: Dovepressmentioning

confidence: 98%

Section: Dovepressmentioning

confidence: 99%

Overview of the pathogenesis and treatment of chronic inflammatory demyelinating polyneuropathy with intravenous immunoglobulins

Mahdi-Rogers

Rajabally²

2010

BTT

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“…No target has definitely been identified, although myelin protein P0 has been suggested. 1 Demyelination is multifocal and can involve the distal nerve terminals, intermediate nerve segments, and nerve roots. Cranial nerves are mildly affected in some patients.…”

Section: Case Historymentioning

confidence: 99%

Chronic inflammatory demyelinating polyradiculoneuropathy with ataxia

Stockdale

Biswas

2004

JRSM

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“…One of the major causes is represented by mutations in the myelin protein zero (P0) 3 gene locus, which leads to Charcot-Marie-Tooth type 1B disorder or Dejerine-Sottas syndrome (1). Heterozygous and homozygous P0-deficient mice serve as animal models for these disorders.…”

mentioning

confidence: 99%

Tolerance Induction by Intrathymic Expression of P0

Visan

Visan²,

Weishaupt

et al. 2004

The Journal of Immunology

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Genetic deficiency or instability of myelin protein zero (P0) results in hereditary motor sensory neuropathy. In view of recent advances in gene therapy, substitution of the molecular defect may become realistic in the near future. Here we investigate the impact of genetic deficiency of P0 on selection of the autoreactive T cell repertoire in the corresponding mouse model. We show that P0 mRNA transcripts are expressed in thymic stroma, similar to other myelin proteins and that expression of intact P0 protein can be detected by Western blot. Using a library of overlapping 20mer peptides spanning the entire length of P0 and applying the ELISPOT technique, we detected a strong immune response toward P0 extracellular domain peptide aa 41–60 in P0−/− knockout mice, but not in heterozygous P0+/− or wild-type (wt) mice. In addition, one cryptic epitope and two subdominant epitopes of P0 were identified. Using P0−/− into wt bone marrow (BM) chimeras we found that P0 expression in the host suffices for full tolerance induction, which is in line with its presence in thymic stroma. However, repopulation of P0−/− mice with wt BM led to partial induction of tolerance, suggesting that BM derived cells can also express this protein. Our findings may have implications for secondary autoimmunity developing after gene therapy in hereditary neuropathies and other diseases with genetically determined protein deficiency, because the repaired protein will then represent a foreign, nontolerized Ag.

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P0 protein is a target antigen in chronic inflammatory demyelinating polyradiculoneuropathy

Cited by 151 publications

References 32 publications

Overview of the pathogenesis and treatment of chronic inflammatory demyelinating polyneuropathy with intravenous immunoglobulins

Overview of the pathogenesis and treatment of chronic inflammatory demyelinating polyneuropathy with intravenous immunoglobulins

Chronic inflammatory demyelinating polyradiculoneuropathy with ataxia

Tolerance Induction by Intrathymic Expression of P0

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