Fibrillin microfibril structure identifies long-range effects of inherited pathogenic mutations affecting a key regulatory TGFβ-binding site
A. R. Godwin, J. Thomson, D. F. Holmes, C. S. Adamo, G. Sengle, M. J. Sherratt, A. M. Roseman, C. Baldock
The paper reveals the cryo-EM structure of fibrillin microfibrils and shows how mutations disrupt TGFβ signaling by blocking LTBP-1 interaction through long-range structural effects.
bioRxiv · Aug 26, 2022 · PreprintBiochemistry