Zeiss Caroline J, Allore Heather G, Beck Amanda P
Section of Comparative Medicine, Yale University School of Medicine, New Haven, Connecticut, United States of America.
Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut, United States of America.
PLoS One. 2017 Feb 9;12(2):e0171790. doi: 10.1371/journal.pone.0171790. eCollection 2017.
Translation of disease-modifying therapies in neurodegenerative disease has been disappointing. Parkinson's disease (PD) was used to compare patterns of preclinical study design for symptomatic and potentially disease-modifying interventions. We examined the relationship of model, intervention type and timing, outcomes and outcome measures in 543 animal and human studies (1973-2015) across a contemporary cohort of animal and human interventional studies (n = 445), animal studies for approved interventions (n = 28), animal and human studies for those that failed to translate (n = 70). Detailed study design data were collected for 216 studies in non-human primate (NHP) and rodent toxin-induced models. Species-specific patterns of study design prevailed regardless of whether interventions were symptomatic or potentially disease-modifying. In humans and NHPs, interventions were typically given to both sexes well after the PD phenotype was established, and clinical outcome measures were collected at single (symptomatic) or multiple (disease-modifying) time-points. In rodents, interventions often preceded induction of the model, acute toxic protocols were common, usually given to young males, clinical outcome measures were used less commonly, and outcomes were less commonly assessed at multiple time points. These patterns were more prevalent in mice than rats. In contrast, study design factors such as randomization and blinding did not differ appreciably across symptomatic and disease-modifying intervention categories. The translational gap for potentially disease-modifying interventions in PD in part results from study designs, particularly in mice, that fail to model the progressive nature and relatively late intervention characteristic of PD, or that anchor mechanistic and neuropathologic data to longitudinal clinical outcomes. Even if measures to improve reproducibility are broadly adopted, perpetuation of these norms will continue to impede effective translation.
神经退行性疾病中疾病修饰疗法的翻译一直令人失望。帕金森病(PD)被用于比较针对症状性和潜在疾病修饰性干预措施的临床前研究设计模式。我们在1973 - 2015年期间的543项动物和人体研究(包括445项当代动物和人体干预研究队列、28项已批准干预措施的动物研究、70项未能转化的干预措施的动物和人体研究)中,研究了模型、干预类型和时间、结果及结果测量之间的关系。收集了216项非人类灵长类动物(NHP)和啮齿动物毒素诱导模型的详细研究设计数据。无论干预措施是症状性的还是潜在疾病修饰性的,特定物种的研究设计模式都占主导地位。在人类和NHP中,干预措施通常在PD表型确立后很久才给予两性,临床结果测量在单一(症状性)或多个(疾病修饰性)时间点进行收集。在啮齿动物中,干预措施通常在模型诱导之前进行,急性毒性方案很常见,通常给予年轻雄性,临床结果测量使用较少,且在多个时间点评估结果的情况也较少。这些模式在小鼠中比在大鼠中更普遍。相比之下,随机化和盲法等研究设计因素在症状性和疾病修饰性干预类别之间没有明显差异。PD中潜在疾病修饰性干预措施的转化差距部分源于研究设计,特别是在小鼠中,这些设计未能模拟PD的渐进性本质和相对较晚的干预特征,或者未能将机制和神经病理学数据与纵向临床结果联系起来。即使广泛采用提高可重复性的措施,这些规范的持续存在仍将继续阻碍有效的转化。