Cook Lola, Verbrugge Jennifer, Schwantes-An Tae-Hwi, Schulze Jeanine, Foroud Tatiana, Hall Anne, Marder Karen S, Mata Ignacio F, Mencacci Niccolò E, Nance Martha A, Schwarzschild Michael A, Simuni Tanya, Bressman Susan, Wills Anne-Marie, Fernandez Hubert H, Litvan Irene, Lyons Kelly E, Shill Holly A, Singer Carlos, Tropea Thomas F, Vanegas Arroyave Nora, Carbonell Janfreisy, Cruz Vicioso Rossy, Katus Linn, Quinn Joseph F, Hodges Priscila D, Meng Yan, Strom Samuel P, Blauwendraat Cornelis, Lohmann Katja, Casaceli Cynthia, Rao Shilpa C, Ghosh Galvelis Kamalini, Naito Anna, Beck James C, Alcalay Roy N
Medical and Molecular Genetics, Indiana University School of Medicine, Indianapolis, IN 46202, USA.
Parkinson's Foundation, NewYork, NY 10018, USA.
Brain. 2024 Aug 1;147(8):2668-2679. doi: 10.1093/brain/awae142.
Variants in seven genes (LRRK2, GBA1, PRKN, SNCA, PINK1, PARK7 and VPS35) have been formally adjudicated as causal contributors to Parkinson's disease; however, individuals with Parkinson's disease are often unaware of their genetic status since clinical testing is infrequently offered. As a result, genetic information is not incorporated into clinical care, and variant-targeted precision medicine trials struggle to enrol people with Parkinson's disease. Understanding the yield of genetic testing using an established gene panel in a large, geographically diverse North American population would help patients, clinicians, clinical researchers, laboratories and insurers better understand the importance of genetics in approaching Parkinson's disease. PD GENEration is an ongoing multi-centre, observational study (NCT04057794, NCT04994015) offering genetic testing with results disclosure and genetic counselling to those in the US (including Puerto Rico), Canada and the Dominican Republic, through local clinical sites or remotely through self-enrolment. DNA samples are analysed by next-generation sequencing including deletion/duplication analysis (Fulgent Genetics) with targeted testing of seven major Parkinson's disease-related genes. Variants classified as pathogenic/likely pathogenic/risk variants are disclosed to all tested participants by either neurologists or genetic counsellors. Demographic and clinical features are collected at baseline visits. Between September 2019 and June 2023, the study enrolled 10 510 participants across >85 centres, with 8301 having received results. Participants were: 59% male; 86% White, 2% Asian, 4% Black/African American, 9% Hispanic/Latino; mean age 67.4 ± 10.8 years. Reportable genetic variants were observed in 13% of all participants, including 18% of participants with one or more 'high risk factors' for a genetic aetiology: early onset (<50 years), high-risk ancestry (Ashkenazi Jewish/Basque/North African Berber), an affected first-degree relative; and, importantly, in 9.1% of people with none of these risk factors. Reportable variants in GBA1 were identified in 7.7% of all participants; 2.4% in LRRK2; 2.1% in PRKN; 0.1% in SNCA; and 0.2% in PINK1, PARK7 or VPS35 combined. Variants in more than one of the seven genes were identified in 0.4% of participants. Approximately 13% of study participants had a reportable genetic variant, with a 9% yield in people with no high-risk factors. This supports the promotion of universal access to genetic testing for Parkinson's disease, as well as therapeutic trials for GBA1 and LRRK2-related Parkinson's disease.
七个基因(LRRK2、GBA1、PRKN、SNCA、PINK1、PARK7和VPS35)中的变异已被正式判定为帕金森病的病因;然而,帕金森病患者往往不知道自己的基因状况,因为临床检测很少进行。因此,基因信息没有纳入临床护理,针对变异的精准医学试验也难以招募到帕金森病患者。了解在北美一个地域广泛的大型人群中使用既定基因检测板进行基因检测的收益,将有助于患者、临床医生、临床研究人员、实验室和保险公司更好地理解遗传学在帕金森病诊疗中的重要性。帕金森病基因世代研究(PD GENEration)是一项正在进行的多中心观察性研究(NCT04057794、NCT04994015),通过当地临床站点或远程自助报名,为美国(包括波多黎各)、加拿大和多米尼加共和国的患者提供基因检测,并披露检测结果和提供遗传咨询。通过下一代测序分析DNA样本,包括缺失/重复分析(Fulgent Genetics),对七个主要帕金森病相关基因进行靶向检测。神经科医生或遗传咨询师会将分类为致病/可能致病/风险变异的结果告知所有接受检测的参与者。在基线访视时收集人口统计学和临床特征。2019年9月至2023年6月期间,该研究在85多个中心招募了10510名参与者,其中8301人收到了检测结果。参与者中:男性占59%;白人占86%,亚洲人占2%,黑人/非裔美国人占4%,西班牙裔/拉丁裔占9%;平均年龄67.4±10.8岁。在所有参与者中,13%观察到可报告的基因变异,包括18%有一个或多个遗传病因“高风险因素”的参与者:早发性(<50岁)、高风险血统(阿什肯纳兹犹太人/巴斯克人/北非柏柏尔人)、一级亲属患病;重要的是,在没有这些风险因素的人群中,这一比例为9.1%。在所有参与者中,7.7%的人检测出GBA1基因有可报告变异;LRRK2基因变异占2.4%;PRKN基因变异占2.1%;SNCA基因变异占0.1%;PINK1、PARK7或VPS35基因变异合计占0.2%。0.4%的参与者检测出七个基因中不止一个基因存在变异。约13%的研究参与者有可报告的基因变异,无高风险因素的人群中这一比例为9%。这支持推广普及帕金森病基因检测,以及针对GBA1和LRRK2相关帕金森病的治疗试验。