From the Department of Immunology (A.J.), CHU Montpellier; Institut de Génomique Fonctionnelle (A.J., J.E.-B., G.T., J.D.), Université de Montpellier, CNRS, INSERM; and Department of Neurology (G.T.), CHU Montpellier, France.
Neurol Neuroimmunol Neuroinflamm. 2025 Jan;12(1):e200336. doi: 10.1212/NXI.0000000000200336. Epub 2024 Nov 18.
Dihydrolipoamide S-acetyltransferase (DLAT), the E2 component of the mitochondrial pyruvate dehydrogenase complex (PDC-E2), has recently been suggested to be a biomarker of chronic inflammatory demyelinating polyneuropathy (CIDP). It was particularly associated with sensory variants of CIDP. Antimitochondrial antibodies are important for the diagnosis of primary biliary cholangitis, but insofar, only 2 studies have reported an association with CIDP. Here, we aimed to validate these observations in a cohort of French patients with immune-mediated neuropathy.
The positivity against PDC-E2/DLAT was examined using ELISA and confirmed using commercially available immuno-DOT and by indirect immunofluorescence on stomach, kidney, and liver sections.
None of the 20 healthy controls, 31 patients with Guillain-Barré syndrome, 102 patients with CIDP (including 24 patients with sensory CIDP), 26 patients with monoclonal gammopathy, 23 patients with Charcot-Marie-Tooth disease, or 20 patients with autoimmune nodopathy showed IgG against PDC-E2/DLAT.
PDC-E2/DLAT is accurately a target antigen in immune-mediated neuropathies.
二氢硫辛酰胺 S-乙酰基转移酶（DLAT）是线粒体丙酮酸脱氢酶复合物（PDC-E2）的 E2 组成部分，最近被认为是慢性炎症性脱髓鞘性多发性神经病（CIDP）的生物标志物。它与 CIDP 的感觉变异尤其相关。抗线粒体抗体对原发性胆汁性胆管炎的诊断很重要，但迄今为止，只有 2 项研究报告了与 CIDP 的关联。在这里，我们旨在验证法国免疫介导性神经病患者队列中的这些观察结果。
使用 ELISA 检查抗 PDC-E2/DLAT 的阳性情况，并使用商业上可用的免疫斑点和胃、肾和肝切片上的间接免疫荧光来确认。
20 名健康对照者、31 名吉兰-巴雷综合征患者、102 名 CIDP 患者（包括 24 名感觉 CIDP 患者）、26 名单克隆丙种球蛋白病患者、23 名夏科-马里-图病患者或 20 名自身免疫性神经病变患者的 IgG 均未针对 PDC-E2/DLAT。
PDC-E2/DLAT 确实是免疫介导性神经病的靶抗原。
