BACKGROUND As a group, fibroproliferative disorders of the lung, liver, kidney, heart, vasculature and integument are common, progressive and refractory to therapy. They can emerge following toxic insults, but are frequently idiopathic. Their enigmatic propensity to resist therapy and progress to organ failure has focused attention on the myofibroblast-the primary effector of the fibroprolifera...

Liver fibrosis results from dysregulation of normal wound healing, inflammation, activation of myofibroblasts, and deposition of extracellular matrix (ECM). Chronic liver injury causes death of hepatocytes and formation of apoptotic bodies, which in turn, release factors that recruit inflammatory cells (neutrophils, monocytes, macrophages, and lymphocytes) to the injured liver. Hepatic macropha...

septum formation. Migration assays performed on primary cell cultures of myofibroblasts from wild type and Rfx3 / lungs show that Rfx3 / myofibroblasts loose their capacities to respond to environmental factors as guidance cues. We are currently dissecting the signaling cascades that are impaired in Rfx3-deficient myofibroblasts. In conclusion, RFX3 regulates primary ciliary growth and governs ...

septum formation. Migration assays performed on primary cell cultures of myofibroblasts from wild type and Rfx3 / lungs show that Rfx3 / myofibroblasts loose their capacities to respond to environmental factors as guidance cues. We are currently dissecting the signaling cascades that are impaired in Rfx3-deficient myofibroblasts. In conclusion, RFX3 regulates primary ciliary growth and governs ...

septum formation. Migration assays performed on primary cell cultures of myofibroblasts from wild type and Rfx3 / lungs show that Rfx3 / myofibroblasts loose their capacities to respond to environmental factors as guidance cues. We are currently dissecting the signaling cascades that are impaired in Rfx3-deficient myofibroblasts. In conclusion, RFX3 regulates primary ciliary growth and governs ...

Bile acids are elevated in the blood of women with intrahepatic cholestasis of pregnancy (ICP) and this may lead to fetal arrhythmia, fetal hypoxia and potentially fetal death in utero. The bile acid taurocholic acid (TC) causes abnormal calcium dynamics and contraction in neonatal rat cardiomyocytes. Ursodeoxycholic acid (UDCA), a drug clinically used to treat ICP, prevents adverse effects of ...

Lung fibrosis is characterized by abnormal accumulation of fibroblasts in the interstitium of the alveolar space. Two populations of myofibroblasts, distinguished by Thy1 expression, are detected in human and murine lungs. Accumulation of Thy1-negative (Thy1(-)) myofibroblasts was shown in the lungs of humans with idiopathic pulmonary fibrosis (IPF) and of bleomycin-treated mice. We aimed to id...

Myofibroblasts are unique contractile cells with both muscle and nonmuscle properties. Typically myofibroblasts are identified by the expression of alpha smooth muscle actin (ASMA); however some myofibroblasts also express sarcomeric proteins. In this study, we show that pulmonary myofibroblasts express three of the eight known sarcomeric myosin heavy chains (MyHCs) (IIa, IId, and embryonic) an...