Secretome analysis of mouse fibroblast

Fibrillin-1, an extracellular matrix (ECM) protein encoded by FBN1 gene, serves as the microfibril scaffold crucial for elastic fiber formation and homeostasis in pliable tissue such as the skin. Besides causing Marfan syndrome, some mutations in FBN1 result in scleroderma, marked by hardened and thicker skin which limits joint mobility. Here, we analyzed the secreted protein profile (secretome) of fibroblast prepared from the Fbn1G234D/G234D mouse model carrying a corresponding variant of FBN1 in the hybrid-1 domain, which was identified in a patient with familial aortic dissection.