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. 2020 Jan 22;11(1):2.
doi: 10.1186/s13317-019-0124-6.

Associations Between HLA and Autoimmune Neurological Diseases With Autoantibodies

Free PMC article

Associations Between HLA and Autoimmune Neurological Diseases With Autoantibodies

Sergio Muñiz-Castrillo et al. Auto Immun Highlights. .
Free PMC article


Recently, several autoimmune neurological diseases have been defined by the presence of autoantibodies against different antigens of the nervous system. These autoantibodies have been demonstrated to be specific and useful biomarkers, and most of them are also pathogenic. These aspects have increased the value of autoantibodies in neurological practice, as they enable to establish more accurate diagnosis and to better understand the underlying mechanisms of the autoimmune neurological diseases when they are compared to those lacking them. Nevertheless, the exact mechanisms leading to the autoimmune response are still obscure. Genetic predisposition is likely to play a role in autoimmunity, HLA being the most reported genetic factor. Herein, we review the current knowledge about associations between HLA and autoimmune neurological diseases with autoantibodies. We report the main alleles and haplotypes, and discuss the clinical and pathogenic implications of these findings.

Keywords: Autoantibodies; Autoimmune encephalitis; HLA; Limbic encephalitis; Myasthenia gravis; Neuromyelitis optica; Paraneoplastic neurological syndrome.

Conflict of interest statement

The authors declare that they have no competing interests.


Fig. 1
Fig. 1
HLA-complex on chromosome 6, showing the main class I and II HLA genes (top). Structure of class I (bottom left) and class II (bottom right) HLA molecules. The alpha chain of class I molecule has three domains (α1–3) and includes the peptide-binding groove. The β2-microglobulin (β2) is not encoded by HLA genes. The class II molecules are made up of one alpha and one beta chain that each contains two domains (α1, α2, β1, β2); both form the peptide-binding groove

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