Loss of cell-autonomously secreted laminin-a2 drives muscle stem cell dysfunction in LAMA2-related muscular dystrophy

The extracellular matrix protein laminin-a2 is essential for preserving the integrity of skeletal muscle fibers during contraction. Its importance is reflected by the severe, congenital LAMA2-related muscular dystrophy (LAMA2 MD) caused by loss-of-function mutations in the LAMA2 gene. While laminin-a2 has an established role in structurally supporting muscle fibers, it remains unclear whether it exerts additional functions that contribute to the maintenance of skeletal muscle integrity. Submitted transcriptomic data represents gene expression profile of control and LAMA2-deficient human myogenic precursor cells derived from induced pluripotent stem cells which was analyzed to better understand the role of laminin-a2 in human cells. Overall design: Human induced pluripotent stem cells (hiPSCs) from a healthy donor in which exon 3 of the LAMA2 gene was deleted using CRISPR/Cas9 method, together with the isogenic control counterparts, were subjected to myogenic differention using Skeletal Muscle Differentiation Kit (Amsbio). RNA was isolated from four independent differentiations at the stage of myogenic precursors and subjected to next generation RNA-sequencing..