Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China.
Neuroscience Center, Chinese Academy of Medical Sciences, Beijing, 100730, China.
Ann Clin Transl Neurol. 2022 Jul;9(7):1027-1038. doi: 10.1002/acn3.51580. Epub 2022 May 18.
Metabolic dysfunction has been suggested to be involved in the pathophysiology of amyotrophic lateral sclerosis (ALS). This study aimed to investigate the potential role of metabolic biomarkers in the progression of ALS and understand the possible metabolic mechanisms.
Fifty-two patients with ALS and 24 normal controls were included, and blood samples were collected for analysis of metabolic biomarkers. Basal anthropometric measures, including body composition and clinical features, were measured in ALS patients. The disease progression rate was calculated using the revised ALS functional rating scale (ALSFRS-R) during the 6-month follow-up.
ALS patients had higher levels of adipokines (adiponectin, adipsin, resistin, and visfatin) and other metabolic biomarkers [C-peptide, glucagon, glucagon-like peptide 1 (GLP-1), gastric inhibitory peptide, and plasminogen activator inhibitor type 1] than controls. Leptin levels in serum were positively correlated with body mass index, body fat, and visceral fat index (VFI). Adiponectin was positively correlated with the VFI and showed a positive correlation with the ALSFRS-R and a negative correlation with baseline disease progression. Patients with lower body fat, VFI, and fat in limbs showed faster disease progression during follow-ups. Lower leptin and adiponectin levels were correlated with faster disease progression. After adjusting for confounders, lower adiponectin levels and higher visfatin levels were independently correlated with faster disease progression.
The current study found altered levels of metabolic biomarkers in ALS patients, which may play a role in ALS pathogenesis. Adiponectin and visfatin represent potential biomarkers for prediction of disease progression in ALS.
代谢功能障碍被认为与肌萎缩侧索硬化症(ALS)的病理生理学有关。本研究旨在探讨代谢生物标志物在 ALS 进展中的潜在作用,并了解可能的代谢机制。
纳入 52 例 ALS 患者和 24 例正常对照者,采集血样分析代谢生物标志物。在 ALS 患者中测量基本人体测量指标,包括身体成分和临床特征。在 6 个月的随访期间,使用修订后的肌萎缩侧索硬化功能评定量表(ALSFRS-R)计算疾病进展率。
与对照组相比,ALS 患者的脂肪因子(脂联素、adipsin、抵抗素和内脂素)和其他代谢生物标志物[C 肽、胰高血糖素、胰高血糖素样肽 1(GLP-1)、胃抑制肽和纤溶酶原激活物抑制剂 1]水平更高。血清瘦素水平与体重指数、体脂肪和内脏脂肪指数(VFI)呈正相关。脂联素与 VFI 呈正相关,与 ALSFRS-R 呈正相关,与基线疾病进展呈负相关。随访期间,体脂肪、VFI 和四肢脂肪较低的患者疾病进展较快。较低的瘦素和脂联素水平与疾病进展较快相关。在调整混杂因素后,较低的脂联素水平和较高的内脂素水平与疾病进展较快独立相关。
本研究发现 ALS 患者代谢生物标志物水平改变,可能在 ALS 发病机制中发挥作用。脂联素和内脂素可能是预测 ALS 疾病进展的潜在生物标志物。