Publications by authors named "Pedro Gameiro Dos Santos"
Article Synopsis
- LAMA2 is a key component of the extracellular matrix (ECM) found primarily in skeletal muscle, and mutations can lead to a severe form of congenital muscular dystrophy known as LAMA2-CMD.
- Research using LAMA2-deficient C2C12 cells revealed issues with muscle cell proliferation, differentiation, and fusion, along with increased DNA damage and mitochondrial dysfunction.
- Findings from fetal myoblasts in a mouse model of LAMA2-CMD indicate that the disease disrupts gene expression in muscle fibers, affecting muscle cell development and responses to oxidative stress and DNA repair.
The extracellular matrix (ECM) plays a crucial role in providing structural support for cells and conveying signals that are important for various cellular processes. Two-dimensional (2D) cell culture models oversimplify the complex interactions between cells and the ECM, as the lack of a complete three-dimensional (3D) support can alter cell behavior, making them inadequate for understanding in vivo processes. Deficiencies in ECM composition and cell-ECM interactions are important contributors to a variety of different diseases.
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