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Paradoxical Changes: EMMPRIN Tissue and Plasma Levels in Marfan Syndrome-Related Thoracic Aortic Aneurysms

Kyle C Alexander1, Carlton W Anderson2, Chris B Agala1

  • 1Department of Surgery, Division of Cardiothoracic Surgery, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

Journal of Clinical Medicine
|March 28, 2024
PubMed

Insights

Extracellular matrix metalloproteinase inducer (EMMPRIN) levels in Marfan syndrome (MFS) thoracic aortic aneurysms (TAAs) differ between tissue and plasma. MT1-MMP influences EMMPRIN secretion, suggesting diagnostic potential for MFS TAAs.

Area of Science:

  • Cardiovascular Biology
  • Extracellular Matrix Dynamics
  • Genetic Connective Tissue Disorders

Background:

  • Marfan syndrome (MFS) associated thoracic aortic aneurysms (TAAs) exhibit unique extracellular matrix metalloproteinase inducer (EMMPRIN) profiles.
  • EMMPRIN shedding into circulation via extracellular vesicles is MT1-MMP dependent.
  • Investigating EMMPRIN and MT1-MMP in MFS TAA tissue and plasma is crucial for understanding these unique profiles.

Purpose of the Study:

  • To investigate the relationship between EMMPRIN and MT1-MMP in MFS TAA tissue and plasma.
  • To determine the mechanism of EMMPRIN secretion in MFS aortic fibroblasts.
  • To explore the diagnostic potential of EMMPRIN and other matrix metalloproteinase analytes in MFS TAAs.

Main Methods:

  • Quantification of protein targets in aortic tissue and plasma from MFS TAA patients and healthy controls.
  • Manipulation of MT1-MMP abundance and localization in aortic fibroblasts.
  • Measurement of secreted EMMPRIN in conditioned culture media.

Main Results:

  • EMMPRIN levels were elevated in MFS TAA tissue but reduced in plasma compared to controls.
  • MT1-MMP and TIMP-2 were elevated in MFS TAA tissue, while MMP-2 and MMP-9 were reduced.
  • EMMPRIN secretion from aortic fibroblasts was dependent on MT1-MMP internalization.

Conclusions:

  • Impaired EMMPRIN secretion, influenced by MT1-MMP cellular localization, contributes to elevated tissue EMMPRIN levels in MFS.
  • Distinct EMMPRIN levels in MFS TAAs, alongside other MMP analytes, differentiate them from controls.
  • These findings suggest potential diagnostic utility for EMMPRIN and related markers in MFS TAAs.
Keywords:
EMMPRINMT1-MMPMarfan syndromethoracic aortic aneurysm

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