Most promisingly, both healthy and scleroderma fibroblasts showed reduced secretion of other abundant fibrotic ECM components, fibrillin1 and fibronectin (Fig. 6A, middle panels and B), further underscoring the importance of TANGO1 in controlling pathophysiological secretion of bulky ECM secretory cargoes. This evidence concerns the gene FN1 and scleroderma.