Anti-NT5C1A autoantibodies for the diagnosis and study of the pathogenesis of sporadic inclusion body myositis
Anti-NT5C1A autoantibodies for the diagnosis and study of the pathogenesis of sporadic inclusion body myositis
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抗NT5C1A自身抗体用于散发性包涵体肌炎的诊断和发病机制研究
DOI：
10.1111/cen3.12420
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发表时间：
2017
影响因子：
--
通讯作者：
Ando Yukio
中科院分区：
文献类型：
--
作者：
Yamashita Satoshi;Tawara Nozomu;Ando Yukio
散发性包涵体肌炎（sIBM）是一种慢性和进行性炎症性肌病，最常见于年龄>50岁的人群。sIBM的特征是股四头肌、手指和腕屈肌的不对称性肌无力和萎缩。sIBM的组织学结果的特征在于炎症和肌变性病理的组合。sIBM的发病机制尚未完全了解，血清标志物尚未确定用于诊断疾病或评估治疗效果。最近，在sIBM患者的血浆和血清样本中发现了抗胞质5′-核苷酸酶1A的自身抗体。已经建立了各种具有临床实用性的方法来检测用于诊断sIBM的抗cN 1A自身抗体。重要的是，自身抗体可能通过引起蛋白酶体和溶酶体降解功能障碍而在疾病的发展中起直接作用。未来的研究应进行阐明的分子机制，肌浆自身抗原的识别和参与肌纤维的变性。进一步的研究是必不可少的，以提供更好地了解sIBM的炎症和退行性过程之间的关系。
Sporadic inclusion body myositis (sIBM) is a chronic and progressive inflammatory myopathy that is commonest in the population aged >50 years. Asymmetric muscle weakness and wasting of the quadriceps, and finger and wrist flexor muscles are characteristic of sIBM. Histological findings for sIBM are characterized by a combination of inflammatory and myodegenerative pathologies. The pathogenesis of sIBM is not yet fully understood, and serum markers have not been identified for diagnosis of the disease or assessment of therapeutic efficacy. Recently, autoantibodies against cytosolic 5′‐nucleotidase 1A have been identified in plasma and serum samples from patients with sIBM. Various methods with clinical utility have been established to detect anti‐cN1A autoantibodies for the diagnosis of sIBM. Importantly, the autoantibodies might have direct roles in the development of the disease by causing dysfunction in proteasomal and lysosomal degradation. Future studies should be carried out to elucidate the molecular mechanisms by which the sarcoplasmic autoantigen is recognized and involved in the degeneration of myofibers. Additional research is essential to provide a better understanding of the relationship between the inflammatory and degenerative processes of sIBM.