Walkley Steven U, Davidson Cristin D, Jacoby Jonathan, Marella Philip D, Ottinger Elizabeth A, Austin Christopher P, Porter Forbes D, Vite Charles H, Ory Daniel S
Dominick P. Purpura Department of Neuroscience, Rose F. Kennedy Intellectual and Developmental Disabilities Research Center, Albert Einstein College of Medicine, 1410 Pelham Parkway South, Bronx, NY, 10461, USA.
Hide and Seek Foundation for Lysosomal Disease Research, 6475 East Pacific Coast Highway, Suite 466, Long Beach, CA, 90803, USA.
Orphanet J Rare Dis. 2016 Dec 1;11(1):161. doi: 10.1186/s13023-016-0540-x.
Rare disease represents one of the most significant issues facing the medical community and health care providers worldwide, yet the majority of these disorders never emerge from their obscurity, drawing little attention from the medical community or the pharmaceutical industry. The challenge therefore is how best to mobilize rare disease stakeholders to enhance basic, translational and clinical research to advance understanding of pathogenesis and accelerate therapy development. Here we describe a rare, fatal brain disorder known as Niemann-Pick type C (NPC) and an innovative research collaborative known as Support of Accelerated Research for NPC (SOAR-NPC) which illustrates one pathway through which knowledge of a rare disease and its possible treatments are being successfully advanced. Use of the "SOAR" mechanism, we believe, offers a blueprint for similar advancement for many other rare disorders.
罕见病是全球医学界和医疗服务提供者面临的最重要问题之一,但这些疾病中的大多数仍鲜为人知,很少引起医学界或制药行业的关注。因此,挑战在于如何最好地动员罕见病相关利益者加强基础、转化和临床研究,以增进对发病机制的理解并加速治疗开发。在此,我们描述一种罕见的致命性脑部疾病——尼曼-匹克C型病(NPC),以及一个名为“NPC加速研究支持”(SOAR-NPC)的创新性研究合作项目,该项目展示了一条成功推进罕见病知识及其可能治疗方法的途径。我们认为,“SOAR”机制的应用为许多其他罕见病的类似进展提供了蓝图。