Diverse effector and regulatory functions of fibro/adipogenic progenitors during skeletal muscle fibrosis in muscular dystrophy
Xingyu Wang, Jianming Chen, Sachiko Homma, Yinhang Wang, Gregory R. Smith, Frederique Ruf-Zamojski, Stuart C. Sealfon, Lan Zhou
Abstract
. The effector and regulatory functions are exerted by diverse FAP clusters which are different between diaphragm and quadriceps, indicating their activation by different tissue microenvironments. FAPs are more abundant in diaphragm than in quadriceps. Our findings suggest that the development of anti-fibrotic therapy for DMD should target not only the ECM production but also the pro-fibrogenic regulatory functions of FAPs.
Topics & Concepts
Extracellular matrixDuchenne muscular dystrophyMuscular dystrophySkeletal muscleCell biologyFibrosisBiologyProgenitor cellDiaphragm (acoustics)AdipogenesisStem cellPathologyMesenchymal stem cellMedicineAnatomyGeneticsPhysicsAcousticsLoudspeakerMuscle Physiology and DisordersAdipose Tissue and MetabolismNeurogenetic and Muscular Disorders Research