PAK proteins and YAP-1 signalling downstream of integrin beta-1 in myofibroblasts promote liver fibrosis

Nat Commun. 2016 Aug 18:7:12502. doi: 10.1038/ncomms12502.

Abstract

Fibrosis due to extracellular matrix (ECM) secretion from myofibroblasts complicates many chronic liver diseases causing scarring and organ failure. Integrin-dependent interaction with scar ECM promotes pro-fibrotic features. However, the pathological intracellular mechanism in liver myofibroblasts is not completely understood, and further insight could enable therapeutic efforts to reverse fibrosis. Here, we show that integrin beta-1, capable of binding integrin alpha-11, regulates the pro-fibrotic phenotype of myofibroblasts. Integrin beta-1 expression is upregulated in pro-fibrotic myofibroblasts in vivo and is required in vitro for production of fibrotic ECM components, myofibroblast proliferation, migration and contraction. Serine/threonine-protein kinase proteins, also known as P21-activated kinase (PAK), and the mechanosensitive factor, Yes-associated protein 1 (YAP-1) are core mediators of pro-fibrotic integrin beta-1 signalling, with YAP-1 capable of perpetuating integrin beta-1 expression. Pharmacological inhibition of either pathway in vivo attenuates liver fibrosis. PAK protein inhibition, in particular, markedly inactivates the pro-fibrotic myofibroblast phenotype, limits scarring from different hepatic insults and represents a new tractable therapeutic target for treating liver fibrosis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adaptor Proteins, Signal Transducing / metabolism*
  • Animals
  • Hepatic Stellate Cells
  • Humans
  • Integrin beta1 / metabolism*
  • Liver Cirrhosis / metabolism*
  • Liver Cirrhosis / pathology*
  • Male
  • Mice, Inbred C57BL
  • Myofibroblasts / metabolism*
  • Myofibroblasts / pathology*
  • Myosin Light Chains / metabolism
  • Phenotype
  • Phosphoproteins / metabolism*
  • Rats, Sprague-Dawley
  • Signal Transduction*
  • Transcription Factors
  • YAP-Signaling Proteins
  • p21-Activated Kinases / metabolism*

Substances

  • Adaptor Proteins, Signal Transducing
  • Integrin beta1
  • Myosin Light Chains
  • Phosphoproteins
  • Transcription Factors
  • YAP-Signaling Proteins
  • YAP1 protein, human
  • p21-Activated Kinases