Soto Marta, Fernández Manel, Bravo Paloma, Lahoz Sara, Garrido Alicia, Sánchez-Rodríguez Antonio, Rivera-Sánchez María, Sierra María, Melón Paula, Roig-García Ana, Naito Anna, Casey Bradford, Camps Jordi, Tolosa Eduardo, Martí María-José, Infante Jon, Ezquerra Mario, Fernández-Santiago Rubén
Lab of Parkinson Disease and Other Neurodegenerative Movement Disorders, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Institut de Neurociències, Universitat de Barcelona, ES-08036, Barcelona, Catalonia, Spain.
Parkinson Disease and Movement Disorders Unit, Neurology Service, Institut Clínic de Neurociències, Hospital Clínic de Barcelona, ES-08036, Barcelona, Catalonia, Spain.
NPJ Parkinsons Dis. 2023 Feb 2;9(1):15. doi: 10.1038/s41531-023-00451-x.
The LRRK2 G2019S pathogenic mutation causes LRRK2-associated Parkinson's disease (L2PD) with incomplete penetrance. LRRK2 non-manifesting carriers (L2NMC) are at PD high risk but predicting pheno-conversion is challenging given the lack of progression biomarkers. To investigate novel biomarkers for PD premotor stages, we performed a longitudinal microRNA (miRNA) assessment of serum samples from G2019S L2NMC followed-up over 8 years. Our cohort consisted of G2019S L2NMC stratified by dopamine transporter single-photon emission computed tomography (DaT-SPECT) into DaT-negative (n = 20) and DaT-positive L2NMC (n = 20), pheno-converted G2019S L2PD patients (n = 20), idiopathic PD (iPD) (n = 19), and controls (n = 40). We also screened a second cohort of L2PD patients (n = 19) and controls (n = 20) (Total n = 158). Compared to healthy controls, we identified eight deregulated miRNAs in DaT-negative L2NMC, six in DaT-positive L2NMC, and one in L2PD. Between groups, the highest miRNA differences, 24 candidate miRNAs, occurred between DaT-positive L2NMC and L2PD. Longitudinally, we found 11 common miRNAs with sustained variation in DaT-negative and DaT-positive L2NMCs compared to their baselines. Our study identifies novel miRNA alterations in premotor stages of PD co-occurring with progressive DaT-SPECT decline before motor manifestation, whose deregulation seems to attenuate after the diagnosis of L2PD. Moreover, we identified four miRNAs with relatively high discriminative ability (AUC = 0.82) between non-pheno-converted DaT-positive G2019S carriers and pheno-converted L2PD patients (miR-4505, miR-8069, miR-6125, and miR-451a), which hold potential as early progression biomarkers for PD.
LRRK2基因的G2019S致病性突变会导致LRRK2相关帕金森病(L2PD),其外显率不完全。LRRK2未表现出症状的携带者(L2NMC)处于帕金森病的高风险中,但由于缺乏疾病进展的生物标志物,预测表型转化具有挑战性。为了研究帕金森病运动前阶段的新型生物标志物,我们对G2019S L2NMC的血清样本进行了长达8年的纵向微小RNA(miRNA)评估。我们的队列包括通过多巴胺转运体单光子发射计算机断层扫描(DaT-SPECT)分层的G2019S L2NMC,分为DaT阴性(n = 20)和DaT阳性L2NMC(n = 20)、已发生表型转化的G2019S L2PD患者(n = 20)、特发性帕金森病(iPD)(n = 19)以及对照组(n = 40)。我们还对另一组L2PD患者(n = 19)和对照组(n = 20)进行了筛查(总样本量n = 158)。与健康对照组相比,我们在DaT阴性L2NMC中鉴定出8种失调的miRNA,在DaT阳性L2NMC中鉴定出6种,在L2PD中鉴定出1种。在各研究组之间,DaT阳性L2NMC和L2PD之间的miRNA差异最大,有24种候选miRNA。纵向研究发现,与基线相比,DaT阴性和DaT阳性L2NMC中有11种常见的miRNA存在持续变化。我们的研究确定了帕金森病运动前阶段与运动症状出现前DaT-SPECT逐渐下降同时出现的新型miRNA改变,在L2PD诊断后这些miRNA的失调似乎有所减轻。此外,我们在未发生表型转化的DaT阳性G2019S携带者和已发生表型转化的L2PD患者之间鉴定出4种具有相对较高鉴别能力(AUC = 0.82)的miRNA(miR-4505、miR-8069、miR-6125和miR-451a),它们有望作为帕金森病早期进展的生物标志物。