| Literature DB >> 31419161 |
Elena M Kaftanovskaya1, Hooi Hooi Ng1, Mariluz Soula1, Bryan Rivas1, Courtney Myhr1, Brian A Ho1, Briana A Cervantes1, Thomas D Shupe2, Mahesh Devarasetty2, Xin Hu3, Xin Xu3, Samarjit Patnaik3, Kenneth J Wilson3, Elena Barnaeva3, Marc Ferrer3, Noel T Southall3, Juan J Marugan3, Colin E Bishop2, Irina U Agoulnik1, Alexander I Agoulnik1.
Abstract
Fibrosis is an underlying cause of cirrhosis and hepatic failure resulting in end stage liver disease with limited pharmacological options. The beneficial effects of relaxin peptide treatment were demonstrated in clinically relevant animal models of liver fibrosis. However, the use of relaxin is problematic because of a short half-life. The aim of this study was to test the therapeutic effects of recently identified small molecule agonists of the human relaxin receptor, relaxin family peptide receptor 1 (RXFP1). The lead compound of this series, ML290, was selected based on its effects on the expression of fibrosis-related genes in primary human stellate cells. RNA sequencing analysis of TGF-β1-activated LX-2 cells showed that ML290 treatment primarily affected extracellular matrix remodeling and cytokine signaling, with expression profiles indicating an antifibrotic effect of ML290. ML290 treatment in human liver organoids with LPS-induced fibrotic phenotype resulted in a significant reduction of type I collagen. The pharmacokinetics of ML290 in mice demonstrated its high stability in vivo, as evidenced by the sustained concentrations of compound in the liver. In mice expressing human RXFP1 gene treated with carbon tetrachloride, ML290 significantly reduced collagen content, α-smooth muscle actin expression, and cell proliferation around portal ducts. In conclusion, ML290 demonstrated antifibrotic effects in liver fibrosis.-Kaftanovskaya, E. M., Ng, H. H., Soula, M., Rivas, B., Myhr, C., Ho, B. A., Cervantes, B. A., Shupe, T. D., Devarasetty, M., Hu, X., Xu, X., Patnaik, S., Wilson, K. J., Barnaeva, E., Ferrer, M., Southall, N. T., Marugan, J. J., Bishop, C. E., Agoulnik, I. U., Agoulnik, A. I. Therapeutic effects of a small molecule agonist of the relaxin receptor ML290 in liver fibrosis.Entities:
Keywords: RXFP1; carbon tetrachloride; collagen; liver organoids
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Year: 2019 PMID: 31419161 PMCID: PMC6988856 DOI: 10.1096/fj.201901046R
Source DB: PubMed Journal: FASEB J ISSN: 0892-6638 Impact factor: 5.191