Published Date: October 24, 2019

Full Text Article

A disease-associated mutation in fibrillin-1 differentially regulates integrin-mediated cell adhesion


Authors: Joselyn S Del Cid, Nilgun Isik Reed, Kathleen Molnar, Sean Liu, Bobo Dang, Sacha A Jensen, William DeGrado, Penny A Handford, Dean Sheppard, Aparna B Sundaram


Fibrillins serve as scaffolds for the assembly of elastic fibers that contribute to the maintenance of tissue homeostasis and regulate growth factor signaling in the extracellular space. Fibrillin-1 is a modular glycoprotein that includes 7 latent transforming growth factor β (TGFβ)-binding protein-like (TB) domains and mediates cell adhesion through integrin binding to the RGD motif in its 4th TB domain. A subset of missense mutations within TB4 cause stiff skin syndrome (SSS), a rare autosomal...



PMID: 31640988DOI: 10.1074/jbc.RA119.011109PMC: PMC6885615