Elevated serum levels of C-terminal agrin fragment in acetylcholine receptor antibody-positive myasthenia gravis

IF 2.9 4区医学 Q3 IMMUNOLOGY

Journal of neuroimmunology Pub Date : 2024-09-10 DOI:10.1016/j.jneuroim.2024.578455

Manato Yasuda , Akiyuki Uzawa , Yosuke Onishi , Hideo Handa , Hiroyuki Akamine , Etsuko Ogaya , Yukiko Ozawa , Hiroki Masuda , Masahiro Mori , Satoshi Kuwabara

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Abstract

Agrin is essential for neuromuscular junction (NMJ) formation and maintenance. The C-terminal agrin fragment (CAF), generated by neurotrypsin-mediated cleavage of agrin, has been gaining attention as a potential biomarker for sarcopenia. We investigated serum CAF levels in myasthenia gravis (MG), a NMJ disorder. Compared to healthy controls, serum CAF levels were significantly elevated in acetylcholine receptor antibody-positive MG (AChR-MG) patients, but not in muscle-specific kinase antibody-positive MG patients. In AChR-MG, baseline and post-treatment CAF levels inversely correlated with post-treatment MG activities of daily living scores, suggesting that elevated CAF levels may reflect protective mechanisms against AChR-MG pathogenesis, such as improved NMJ regeneration.

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乙酰胆碱受体抗体阳性的重症肌无力患者血清中 C 端激动素片段水平升高

神经肌肉接头（NMJ）的形成和维持离不开 Agrin。由神经胰蛋白酶介导的胰蛋白酶裂解产生的 C 端胰蛋白酶片段（CAF）作为一种潜在的肌肉疏松症生物标志物受到越来越多的关注。我们研究了重症肌无力（MG）这种 NMJ 疾病的血清 CAF 水平。与健康对照组相比，乙酰胆碱受体抗体阳性的 MG（AChR-MG）患者的血清 CAF 水平明显升高，而肌肉特异性激酶抗体阳性的 MG 患者的血清 CAF 水平则没有升高。在乙酰胆碱受体抗体阳性的 MG 患者中，基线和治疗后的 CAF 水平与治疗后的 MG 日常活动评分成反比，这表明 CAF 水平的升高可能反映了针对乙酰胆碱受体抗体阳性 MG 发病机制的保护机制，如改善 NMJ 再生。

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来源期刊

Journal of neuroimmunology 医学-免疫学

CiteScore

6.10

自引率

3.00%

发文量

154

审稿时长

37 days

期刊介绍： The Journal of Neuroimmunology affords a forum for the publication of works applying immunologic methodology to the furtherance of the neurological sciences. Studies on all branches of the neurosciences, particularly fundamental and applied neurobiology, neurology, neuropathology, neurochemistry, neurovirology, neuroendocrinology, neuromuscular research, neuropharmacology and psychology, which involve either immunologic methodology (e.g. immunocytochemistry) or fundamental immunology (e.g. antibody and lymphocyte assays), are considered for publication.