顱縫再生可減輕顱骨融合症的顱骨和神經認知缺陷
作者：
小柯機器人發布時間：2021/1/9 21:15:04
美國南加利福尼亞大學顱面分子生物學中心Yang Chai研究組近日取得一項新成果。經過不懈努力，他們的研究發現顱縫再生緩解了顱骨融合症的顱骨和神經認知缺陷。2021年1月7日出版的《細胞》雜誌發表了這項成果。
他們顯示了具有顱骨突觸的Twist1 +/-小鼠具有顱內壓升高和神經認知行為異常表型，重現了人類Saethre-Chotzen症候群的特徵。使用可生物降解的材料與MSC結合，他們成功地再生了功能性顱骨縫合線，可糾正顱骨畸形，使顱內壓正常化並挽救神經認知行為缺陷。再生的縫線創造了一個內生的間充質幹細胞（MSC）遷移到的位置，維持了顱骨的穩態和修復。基於MSC的顱骨縫線再生為這種破壞性疾病中的顱骨和神經認知異常逆轉提供了治療模式的轉變。
據了解，顱骨融合症是由顱縫線過早融合引起的，顱骨縫線包含MSC，這對顱蓋擴張與腦部生長協調至關重要。患有顱骨融合的嬰兒具有顱骨畸形、顱內壓增高以及併發症，例如損害生活質量的神經認知障礙。缺乏概括這些表型的動物模型，阻礙了迫切需要的創新療法的發展。
附：英文原文
Title: Cranial Suture Regeneration Mitigates Skull and Neurocognitive Defects in Craniosynostosis
Author: Mengfei Yu, Li Ma, Yuan Yuan, Xin Ye, Axel Montagne, Jinzhi He, Thach-Vu Ho, Yingxi Wu, Zhen Zhao, Naomi Sta Maria, Russell Jacobs, Mark Urata, Huiming Wang, Berislav V. Zlokovic, Jian-Fu Chen, Yang Chai
Issue&Volume: 2021/01/07
Abstract: Craniosynostosis results from premature fusion of the cranial suture(s), which containmesenchymal stem cells (MSCs) that are crucial for calvarial expansion in coordinationwith brain growth. Infants with craniosynostosis have skull dysmorphology, increasedintracranial pressure, and complications such as neurocognitive impairment that compromisequality of life. Animal models recapitulating these phenotypes are lacking, hamperingdevelopment of urgently needed innovative therapies. Here, we show that Twist1+/ mice with craniosynostosis have increased intracranial pressure and neurocognitivebehavioral abnormalities, recapitulating features of human Saethre-Chotzen syndrome.Using a biodegradable material combined with MSCs, we successfully regenerated a functionalcranial suture that corrects skull deformity, normalizes intracranial pressure, andrescues neurocognitive behavior deficits. The regenerated suture creates a niche intowhich endogenous MSCs migrated, sustaining calvarial bone homeostasis and repair.MSC-based cranial suture regeneration offers a paradigm shift in treatment to reverseskull and neurocognitive abnormalities in this devastating disease.
DOI: 10.1016/j.cell.2020.11.037
Source: https://www.cell.com/cell/fulltext/S0092-8674(20)31609-3
Cell：《細胞》，創刊於1974年。隸屬於細胞出版社，最新IF：36.216