Chen Junchen, Hadi Fatemeh, Wen Xingzhao, Zhao Wenxin, Xu Ming, Xue Shuanghong, Lin Pei, Calandrelli Riccardo, Richard John Lalith Charles, Song Zhixuan, Li Jessica, Amani Alborz, Liu Yang, Chen Xu, Zhong Sheng
Shu Chien-Gene Lay Department of Bioengineering, University of California, San Diego, La Jolla, San Diego, CA, USA.
Institute of Engineering in Medicine, University of California, San Diego, La Jolla, San Diego, CA, USA.
Cell. 2025 Jun 26;188(13):3513-3529.e26. doi: 10.1016/j.cell.2025.03.045. Epub 2025 Apr 23.
Virtually all individuals aged 65 or older develop at least early pathology of Alzheimer's disease (AD), yet most lack disease-causing mutations in APP, PSEN, or MAPT, and many do not carry the APOE4 risk allele. This raises questions about AD development in the general population. Although transcriptional dysregulation has not traditionally been a hallmark of AD, recent studies reveal significant epigenomic changes in late-onset AD (LOAD) patients. We show that altered expression of the LOAD biomarker phosphoglycerate dehydrogenase (PHGDH) modulates AD pathology in mice and human brain organoids independent of its enzymatic activity. PHGDH has an uncharacterized role in transcriptional regulation, promoting the transcription of inhibitor of nuclear factor kappa-B kinase subunit alpha (IKKa) and high-mobility group box 1 (HMGB1) in astrocytes, which suppress autophagy and accelerate amyloid pathology. A blood-brain-barrier-permeable small-molecule inhibitor targeting PHGDH's transcriptional function reduces amyloid pathology and improves AD-related behavioral deficits. These findings highlight transcriptional regulation in LOAD and suggest therapeutic strategies beyond targeting familial mutations.
几乎所有65岁及以上的个体都会出现至少早期的阿尔茨海默病(AD)病理特征,但大多数人在淀粉样前体蛋白(APP)、早老素(PSEN)或微管相关蛋白tau(MAPT)中没有致病突变,而且许多人也不携带APOE4风险等位基因。这引发了关于普通人群中AD发病机制的问题。虽然转录失调传统上并非AD的标志性特征,但最近的研究揭示了晚发性AD(LOAD)患者存在显著的表观基因组变化。我们发现,LOAD生物标志物磷酸甘油酸脱氢酶(PHGDH)的表达改变可调节小鼠和人脑类器官中的AD病理,且与其酶活性无关。PHGDH在转录调控中具有尚未明确的作用,它能促进星形胶质细胞中核因子κB激酶亚基α(IKKα)和高迁移率族蛋白B1(HMGB1)的转录,而这两种蛋白会抑制自噬并加速淀粉样病理过程。一种靶向PHGDH转录功能的可透过血脑屏障的小分子抑制剂可减轻淀粉样病理并改善与AD相关的行为缺陷。这些发现凸显了LOAD中的转录调控作用,并提示了超越针对家族性突变的治疗策略。