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Fatty Acid and Carnitine Metabolism Are Dysregulated in Systemic Sclerosis Patients

A Ottria1,2, A T Hoekstra3, M Zimmermann1,2

  • 1Center for Translational Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, Netherlands.

Frontiers in Immunology
|June 13, 2020
PubMed

Insights

Systemic sclerosis patients show altered carnitine and fatty acid metabolism in immune cells and plasma. Inhibiting carnitine transport reduces inflammation, suggesting metabolic targets for SSc treatment.

Area of Science:

  • Immunology
  • Metabolomics
  • Systemic Sclerosis (SSc) research

Background:

  • Systemic sclerosis (SSc) is a rare, chronic autoimmune disease with unknown causes, marked by fibrosis and immune system dysfunction.
  • Cellular metabolism's role in immune system regulation and disease pathogenesis is a growing area of research interest.

Purpose of the Study:

  • To investigate the metabolic status of plasma and dendritic cells (DCs) in SSc patients.
  • To explore the link between metabolic alterations, specifically carnitine and fatty acids, and inflammation in SSc.

Main Methods:

  • Metabolomic analysis of plasma and dendritic cells from SSc patients.
  • Validation of carnitine alterations in independent patient cohorts.
  • Experimental inhibition of carnitine transport in dendritic cells using etoposide.

Main Results:

  • A distinct dysregulated metabolomic signature of carnitine was identified in both plasma and DCs of SSc patients.
  • Carnitine alterations were confirmed across multiple independent measurements and cohorts.
  • Inhibition of carnitine transport in dendritic cells suppressed pro-inflammatory cytokine (e.g., IL-6) production by reducing fatty acid oxidation.

Conclusions:

  • Altered carnitine and fatty acid metabolism are implicated in the immune system's dysregulation in SSc.
  • Targeting carnitine transport may represent a novel therapeutic strategy to mitigate inflammation in SSc.
Keywords:
carnitinesdendritic cellsfatty acid oxidationmetabolomicssystemic sclerosis

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