Whole mount of adult ear skin as a model to study vascular malformations

作     者：Brandee Decker-Rockefeller Qingfen Li Kevin Pumiglia Brandee Decker-Rockefeller;Qingfen Li;Kevin Pumiglia

作者机构：Biology DepartmentUtica UniversityUticaNew YorkUSA Department of Regenerative and Cancer Cell BiologyAlbany Medical CollegeAlbanyNew YorkUSA 

出 版 物：《Animal Models and Experimental Medicine》 (动物模型与实验医学（英文）)

年 卷 期：2023年第6卷第4期

页      面：362-368页

核心收录：

学科分类：1002[医学-临床医学] 100201[医学-内科学(含：心血管病、血液病、呼吸系病、消化系病、内分泌与代谢病、肾病、风湿病、传染病)] 10[医学] 

基　　金：David E.Bryant Trust Leopold Fund for Vascular Anomalies National Institutes of Health,Grant/Award Number:1R01HL151679 

主　　题：animal disease models pathological angiogenesis PIK3CA rapamycin vascular malformations 

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 ***:We have developed a mouse model with inducible and endothelial-specific expression of PIK3CA H1047R,resulting in the development of vascular *** 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 ***:This approach allows us to successfully model the human disease in a mature and established vascular bed and track the development of vascular *** validate the utility of this model,we applied a topical rapamycin ointment,as rapamycin is therapeutically beneficial to patients in clinical *** found that the induced ear lesions showed significant attenuation after treatment,which was easily ***:These data collectively provide evidence of a new model to study vascular malformations in adult tissues,which should be particularly useful in environments lacking specialized small-animal imaging facilities.