Background Hutchinson-Gilford progeria syndrome(HGPS) is a rare disorder characterized by premature aging and death mainly because of myocardial infarction, stroke, or heart failure. Patients with HGPS are healthy at ...Background Hutchinson-Gilford progeria syndrome(HGPS) is a rare disorder characterized by premature aging and death mainly because of myocardial infarction, stroke, or heart failure. Patients with HGPS are healthy at birth, then get growth impairment, such as loss of subcutaneous fat, alopecia, osteoporosis and heart diseases in 1-2years. HGPS is caused by progerin, which is a toxic form of lamin A expressed in most differentiated cells. Here, we discuss current views about the molecular mechanisms, the mouse models and the treatment approaches of HGPS.We summarize the work in this area and provide directions and clues for future studies.展开更多
基金sponsored by grants from the National key R&D Program of China (No. 2018YFA0108700,No. 2017YFA0105602, No. 2018YFA0108100)National Natural Science Foundation of China (No. 81720108004, NO. 81974019, No. 31871474)+4 种基金the Research Team Project of Natural Science Foundation of Guangdong Province of China (No. 2017A030312007)Science and Technology Planning Project of Guangdong Province (No. 2022B1212010010)the Key Program of Guangzhou Science Research Plan (No. 201904020047)The Special Project of Dengfeng Program of Guangdong Provincial People's Hospital (No. DFJH201812, NO. KJ012019 119, No.KJ012019423)the Shanghai Tech University start-up fund。
文摘Background Hutchinson-Gilford progeria syndrome(HGPS) is a rare disorder characterized by premature aging and death mainly because of myocardial infarction, stroke, or heart failure. Patients with HGPS are healthy at birth, then get growth impairment, such as loss of subcutaneous fat, alopecia, osteoporosis and heart diseases in 1-2years. HGPS is caused by progerin, which is a toxic form of lamin A expressed in most differentiated cells. Here, we discuss current views about the molecular mechanisms, the mouse models and the treatment approaches of HGPS.We summarize the work in this area and provide directions and clues for future studies.
