Alzheon, Inc., 111 Speen Street, Suite 306, Framingham, MA, 01701, USA.
University of Rochester School of Medicine and Dentistry, Rochester, NY, USA.
Alzheimers Res Ther. 2020 Aug 12;12(1):95. doi: 10.1186/s13195-020-00663-w.
The body of evidence suggesting a causative, initiating role of beta amyloid (Aβ) in the pathogenesis of Alzheimer's disease (AD) is substantial. Yet, only a few anti-amyloid agents have shown meaningful efficacy in clinical trials. We evaluated the unifying characteristics of anti-amyloid agents with positive clinical or biomarker effects in long-duration trials and analyzed how pharmacological characteristics determine their clinical product profiles. Four agents with the potential for near term approval fulfill these criteria: the injectable antibodies, aducanumab, gantenerumab, and BAN2401, and a small molecule oral agent, ALZ-801. Aducanumab and BAN2401 showed significant efficacy on both clinical and biomarker outcomes; gantenerumab showed significant biomarker effects, with no clinical efficacy reported to date; and ALZ-801 showed significant clinical effects in the high-risk population of patients homozygous for the ε4 allele of apolipoprotein E gene (APOE4) and a dose-dependent preservation of hippocampal volume. We explored how the pharmacological properties of these agents, namely selectivity for Aβ oligomers, plasma half-life, brain penetration, and time to peak brain exposure, determine their clinical profiles. A crucial characteristic shared by these agents is their ability to engage neurotoxic soluble Aβ oligomers, albeit to various degrees. Aducanumab and gantenerumab partially target oligomers, while mostly clearing insoluble amyloid plaques; BAN2401 preferentially targets soluble protofibrils (large oligomers) over plaques; and ALZ-801 blocks the formation of oligomers without binding to plaques. The degree of selectivity for Aβ oligomers and brain exposure drive the magnitude and onset of clinical efficacy, while the clearance of plaques is associated with vasogenic brain edema. Only the highest doses of aducanumab and BAN2401 show modest efficacy, and higher dosing is limited by increased risk of vasogenic edema, especially in APOE4 carriers. These limitations can be avoided, and efficacy improved by small molecule agents that selectively inhibit the formation or block the toxicity of Aβ oligomers without clearing amyloid plaques. The most advanced selective anti-oligomer agent is ALZ-801, an optimized oral prodrug of tramiprosate, which demonstrated efficacy in homozygous APOE4/4 AD subjects. ALZ-801 selectively and fully inhibits the formation of Aβ42 oligomers at the clinical dose, without evidence of vasogenic edema, and will be evaluated in a phase 3 trial in homozygous APOE4/4 patients with early AD. In addition to clinical measures, the phase 3 trial will include cerebrospinal fluid, plasma, and imaging biomarkers to gain further insights into the role of soluble Aβ oligomers in the pathogenesis of AD and their impact on disease progression.
大量证据表明β淀粉样蛋白(Aβ)在阿尔茨海默病(AD)发病机制中具有因果关系和起始作用。然而,只有少数抗淀粉样蛋白药物在临床试验中显示出有意义的疗效。我们评估了在长期临床试验中具有阳性临床或生物标志物效果的抗淀粉样蛋白药物的统一特征,并分析了药理特性如何决定其临床产品特征。有四种具有近期批准潜力的药物符合这些标准:可注射抗体 aducanumab、gantenerumab、BAN2401 和一种小分子口服药物 ALZ-801。aducanumab 和 BAN2401 在临床和生物标志物结果上均显示出显著疗效;gantenerumab 显示出显著的生物标志物效果,但迄今为止尚未报告临床疗效;ALZ-801 在载脂蛋白 E 基因(APOE4)纯合子高危人群中显示出显著的临床效果,并具有剂量依赖性的海马体积保留。我们探讨了这些药物的药理特性,即对 Aβ 寡聚体的选择性、血浆半衰期、脑穿透性和达到脑暴露峰值的时间,如何决定其临床特征。这些药物的一个关键共同特征是它们能够与神经毒性可溶性 Aβ 寡聚体结合,尽管程度不同。aducanumab 和 gantenerumab 部分靶向寡聚体,而主要清除不溶性淀粉样斑块;BAN2401 优先靶向可溶性原纤维(大寡聚体)而不是斑块;ALZ-801 阻断寡聚体的形成而不与斑块结合。对 Aβ 寡聚体的选择性和脑暴露程度决定了临床疗效的程度和开始时间,而斑块的清除与血管源性脑水肿有关。只有最高剂量的 aducanumab 和 BAN2401 显示出适度的疗效,更高的剂量受到血管源性水肿风险增加的限制,尤其是在 APOE4 携带者中。这些限制可以通过选择性抑制 Aβ 寡聚体形成或阻断其毒性而不清除淀粉样斑块的小分子药物来避免,并提高疗效。最先进的选择性抗寡聚体药物是 ALZ-801,它是 tramiprosate 的优化口服前药,在纯合子 APOE4/4 AD 受试者中显示出疗效。ALZ-801 在临床剂量下选择性和完全抑制 Aβ42 寡聚体的形成,没有血管源性水肿的证据,并将在伴有早期 AD 的纯合子 APOE4/4 患者的 3 期试验中进行评估。除了临床措施外,3 期试验还将包括脑脊液、血浆和影像学生物标志物,以进一步了解可溶性 Aβ 寡聚体在 AD 发病机制中的作用及其对疾病进展的影响。