In addition to Col1a1 transcription and myofibroblast differentiation, TGFβ1, through its type 1 kinase receptor, is largely responsible for the upkeep of Plod2 activation in fibrotic fibroblasts during idiopathic pulmonary fibrosis, keloid formation, interstitial kidney disease, cirrhosis, and scleroderma [11, 18, 23, 36, 43] to encode for the various LHs that catalyze Pyd [46]. Here, TGFB1 is linked to scleroderma.