DOI: 10.1002/ame2.12343
Whole mount of adult ear skin as a model to study vascular malformations
Brandee Decker-Rockefeller1
Qingfen Li2
Kevin Pumiglia2
1.Biology Department,Utica University,Utica,New York,USA2.Department of Regenerative and Cancer Cell Biology,Albany Medical College,Albany,New York,USA
摘要：Background:Genetic analysis in human patients has linked mutations in PIK3CA,the catalytic subunit of PI-3′Kinase,to sporadic incidences of vascular malformations.Methods:We have developed a mouse model with inducible and endothelial-specific expression of PIK3CA H1047R,resulting in the development of vascular malformations.Systemic induction of this mutation in adult mice results in rapid lethality,limiting our ability to track and study these lesions;therefore,we developed a topical and local induction protocol using the active metabolite of tamoxifen,4OH-T,on the ear skin of adults.Results:This approach allows us to successfully model the human disease in a mature and established vascular bed and track the development of vascular malformations.To validate the utility of this model,we applied a topical rapamycin ointment,as ra-pamycin is therapeutically beneficial to patients in clinical trials.We found that the induced ear lesions showed significant attenuation after treatment,which was easily quantified.Conclusions:These data collectively provide evidence of a new model to study vascu-lar malformations in adult tissues,which should be particularly useful in environments lacking specialized small-animal imaging facilities.
机标关键词：studyadultmodelmalformationsmountskinvascularwhole
论文发表日期：2023-08-28
在线出版日期：2025-08-15（本平台首次上网日期，不代表文献的发表时间）
页数：7( 362-368 )
英文信息