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Review
. 2023 Jul 18;43(1):39.
doi: 10.1186/s41232-023-00291-5.

Autoimmune-mediated astrocytopathy

Makoto Kinoshita  1 , Tatsusada Okuno  2
Affiliations
Review

Autoimmune-mediated astrocytopathy

Makoto Kinoshita et al. Inflamm Regen. .

Abstract

Recently accumulating evidence identified the disease entity where astrocytes residing within the central nervous system (CNS) are the target of autoantibody-mediated autoimmunity. Aquaporin4 (AQP4) is the most common antigen to serve as astrocyte-targeted autoimmune responses. Here, in this review, the clinical and pathological aspects of AQP4-mediated astrocyte disease are discussed together with the pathogenic role of anti-AQP4 antibody. More recently, the mechanism of immune dysregulation resulting in the production of astrocyte-targeted autoantibody is also revealed, and the postulated hypothesis is discussed.

Keywords: AQP4; Astrocyte; Autoimmunity.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
The pathogenicity of AQP4-IgG. The destruction of BBB induced by CNS-infiltrating T cells allows AQP4-IgG produced by B cells in peripheral blood to get an access to AQP4 expressed on astrocytes, leading to complement-dependent astrocyte damage. Figures were created with biorender.com
Fig. 2
Fig. 2
Identification of the source of cell-free DNA by cell type-specific methylation patterns. Figures were created with biorender.com
See this image and copyright information in PMC

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