Institut de la Vision, Sorbonne Université, INSERM, CNRS, Paris, France.
Department of Ophthalmology, Hôpital Lariboisière, AP-HP, Université de Paris, Paris, France.
Glia. 2021 Jul;69(7):1679-1693. doi: 10.1002/glia.23983. Epub 2021 Mar 8.
Muller glial cells (MGCs) are responsible for the homeostatic and metabolic support of the retina. Despite the importance of MGCs in retinal disorders, reliable and accessible human cell sources to be used to model MGC-associated diseases are lacking. Although primary human MGCs (pMGCs) can be purified from post-mortem retinal tissues, the donor scarcity limits their use. To overcome this problem, we developed a protocol to generate and bank human induced pluripotent stem cell-derived MGCs (hiMGCs). Using a transcriptome analysis, we showed that the three genetically independent hiMGCs generated were homogeneous and showed phenotypic characteristics and transcriptomic profile of pMGCs. These cells expressed key MGC markers, including Vimentin, CLU, DKK3, SOX9, SOX2, S100A16, ITGB1, and CD44 and could be cultured up to passage 8. Under our culture conditions, hiMGCs and pMGCs expressed low transcript levels of RLPB1, AQP4, KCNJ1, KCJN10, and SLC1A3. Using a disease modeling approach, we showed that hiMGCs could be used to model the features of diabetic retinopathy (DR)-associated dyslipidemia. Indeed, palmitate, a major free fatty acid with elevated plasma levels in diabetic patients, induced the expression of inflammatory cytokines found in the ocular fluid of DR patients such as CXCL8 (IL-8) and ANGPTL4. Moreover, the analysis of palmitate-treated hiMGC secretome showed an upregulation of proangiogenic factors strongly related to DR, including ANG2, Endoglin, IL-1β, CXCL8, MMP-9, PDGF-AA, and VEGF. Thus, hiMGCs could be an alternative to pMGCs and an extremely valuable tool to help to understand and model glial cell involvement in retinal disorders, including DR.
Muller 胶质细胞(MGCs)负责视网膜的稳态和代谢支持。尽管 MGCs 在视网膜疾病中非常重要,但缺乏可靠且易于获得的用于模拟与 MGC 相关疾病的人类细胞来源。虽然可以从死后的视网膜组织中纯化出原代人 MGC(pMGC),但供体的缺乏限制了其使用。为了克服这个问题,我们开发了一种生成和储存人诱导多能干细胞衍生的 MGC(hiMGC)的方案。通过转录组分析,我们表明生成的三个独立的遗传 hiMGC 是同质的,表现出 pMGC 的表型特征和转录组特征。这些细胞表达关键的 MGC 标志物,包括波形蛋白、CLU、DKK3、SOX9、SOX2、S100A16、ITGB1 和 CD44,并且可以培养至第 8 代。在我们的培养条件下,hiMGC 和 pMGC 表达低水平的 RLPB1、AQP4、KCNJ1、KCJN10 和 SLC1A3。通过疾病建模方法,我们表明 hiMGC 可用于模拟糖尿病性视网膜病变(DR)相关血脂异常的特征。事实上,棕榈酸是一种在糖尿病患者中血浆水平升高的主要游离脂肪酸,可诱导 DR 患者眼液中发现的炎症细胞因子的表达,如 CXCL8(IL-8)和 ANGPTL4。此外,对棕榈酸处理的 hiMGC 分泌组的分析表明,与 DR 强烈相关的促血管生成因子的表达上调,包括 ANG2、Endoglin、IL-1β、CXCL8、MMP-9、PDGF-AA 和 VEGF。因此,hiMGC 可以替代 pMGC,并成为一种非常有价值的工具,有助于理解和模拟胶质细胞参与视网膜疾病,包括 DR。