Schnurri-3 inhibition rescues skeletal fragility and vascular skeletal stem cell niche pathology in the OIM model of osteogenesis imperfecta  

作　　者：Na Li Baohong Shi Zan Li Jie Han Jun Sun Haitao Huang Alisha R.Yallowitz Seoyeon Bok Shuang Xiao Zuoxing Wu Yu Chen Yan Xu Tian Qin Rui Huang Haiping Zheng Rong Shen Lin Meng Matthew B.Greenblatt Ren Xu 

机构地区：[1]State Key Laboratory of Cellular Stress Biology,Cancer Research Center,School of Medicine,Faculty of Medicine and Life Sciences,Xiamen University,Xiamen 361102,China [2]Fujian Provincial Key Laboratory of Organ and Tissue Regeneration,School of Medicine,Xiamen University,Xiamen 361102,China [3]Department of Sports Medicine,Xiangya Hospital,Central South University,Changsha 410000,China [4]Research Division,Hospital for Special Surgery,New York,NY 10065,USA [5]Department of Pathology and Laboratory Medicine,Weill Cornell Medical College,New York,NY 10065,USA [6]Department of Electronic and Computer Engineering,Ritsumeikan University,Kusatsu,Shiga 525-8577,Japan

出　　处：《Bone Research》2024年第3期675-688,共14页骨研究（英文版）

基　　金：supported by the National Natural Science Foundation of China (81972034,92068104 and 82002262 to R.X.);the National Key R&D Program of China (2020YFA0112900 to R.X.);Project of Xiamen Cell Therapy Research Center (3502Z20214001 to R.X.);supported by a the NIH grant of US (R01AR075585,R01HD115274,R01CA282815 to M.B.G.);Career Award for Medical Scientists from the Burroughs Wellcome Fund;a Pershing Square Sohn Cancer Research Alliance and the Maximizing Innovation in Neuroscience Discovery (MIND)Prize;supported by a Jump Start Research Career Development Award from Weill Cornell Medicine;a Study Abroad Scholarships from the Mogam Science Scholarship Foundation。

摘　　要：Osteogenesis imperfecta(OI)is a disorder of low bone mass and increased fracture risk due to a range of genetic variants that prominently include mutations in genes encoding typeⅠcollagen.While it is well known that OI reflects defects in the activity of bone-forming osteoblasts,it is currently unclear whether OI also reflects defects in the many other cell types comprising bone,including defects in skeletal vascular endothelium or the skeletal stem cell populations that give rise to osteoblasts and whether correcting these broader defects could have therapeutic utility.

关 键 词：SKELETAL imperfect ENDOTHELIUM 

分 类 号：R681.1[医药卫生—骨科学]