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Mannose Phosphate Isomerase and Mannose Regulate Hepatic Stellate Cell Activation and Fibrosis in Zebrafish and

Charles DeRossi1, Kathryn Bambino2, Joshua Morrison1

  • 1Department of Pediatrics, Icahn School of Medicine at Mount Sinai, New York, NY.

Hepatology (Baltimore, Md.)
|April 25, 2019
PubMed

Insights

Mannose phosphate isomerase (MPI) deficiency causes liver fibrosis. Mannose supplementation can reduce liver fibrosis by inhibiting hepatic stellate cell activation, offering a potential therapeutic strategy.

Area of Science:

  • Biochemistry
  • Hepatology
  • Molecular Biology

Background:

  • Liver fibrosis is a significant health concern with limited treatment options.
  • A rare genetic disorder linked to mannose phosphate isomerase (MPI) mutations causes liver fibrosis in children.
  • Understanding MPI's role in liver health is crucial for developing new therapies.

Purpose of the Study:

  • To investigate the function of MPI and mannose metabolism in liver development and disease.
  • To explore MPI's role in chronic liver diseases and hepatic fibrosis.
  • To assess the therapeutic potential of modulating mannose metabolism.

Main Methods:

  • Analysis of transcriptomic data from human liver cohorts.
  • MPI depletion in zebrafish liver and human hepatic stellate cell (HSC) lines.
  • Assessment of fibrotic responses and HSC activation.
  • Mannose supplementation in various models of liver fibrosis.

Main Results:

  • MPI gene expression is decreased proportionally to fibrosis severity in chronic liver diseases.
  • MPI depletion in vivo and in vitro activates fibrotic responses and promotes HSC activation.
  • Mannose supplementation effectively reduces HSC activation and fibrotic markers in multiple models.

Conclusions:

  • Loss of MPI function promotes hepatic stellate cell activation and liver fibrosis.
  • Modulating mannose metabolism pathways presents a promising therapeutic approach for liver fibrosis.
  • Mannose supplementation could be a viable strategy to reduce HSC activation and improve liver fibrosis outcomes.

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