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The transduction of TGF-β pathway signaling. A: the normal fibrillin-1 as a structural component of extracellular matrix microfibrils participates in the matrix sequestering of TGF-β. B: mutant fibrillin-1 loss interactions with LTBP, hence increasing the concentration of TGF-β in the extracellular matrix and upregulating the TGF-β pathway signal.
