Peroxisomal catalase and plasmalogen biosynthesis protect from oxidative stress in Barth syndrome cardiomyopathy - Report - MDSpire

Peroxisomal catalase and plasmalogen biosynthesis protect from oxidative stress in Barth syndrome cardiomyopathy

  • By

  • Elsie Kajese

  • Malte Hachmann

  • Katharina J. Ermer

  • Manuela Erk

  • Lin Alhasaan

  • Michael Kohlhaas

  • Heike Bömmel

  • Lisa Berberich

  • Christopher Carlein

  • Hanna Eberl

  • Katrin Streckfuß-Bömeke

  • Leticia Prates Roma

  • Süleyman Ergün

  • Christoph Maack

  • Srikanth Karnati

  • Jan Dudek

  • June 30, 2026

  • 0 min

Share

Role of Peroxisomal Catalase and Plasmalogen Synthesis in Mitigating Oxidative Stress in Barth Syndrome-Related Cardiomyopathy

Background

Barth syndrome (BTHS) is a mitochondrial disorder that leads to significant cardiac and metabolic dysfunctions. Understanding the underlying mechanisms of oxidative stress in BTHS is crucial for developing targeted therapies. The interplay between peroxisomes and mitochondria is particularly relevant in the context of fatty acid metabolism and redox control.

Data Highlights

No numerical data or trial data presented in the source material.

Key Findings

  • Barth syndrome is caused by mutations in the TAFAZZIN gene, leading to cardiomyopathy and metabolic dysfunction.
  • Taz-deficiency affects mitochondrial lipid composition, impacting energy conversion and substrate metabolism.
  • Mitochondrial Ca2+ uniporter (MCU) deficiency in Taz-KD hearts compromises redox regulation and contributes to arrhythmias.
  • Metabolic compensation in BTHS involves increased glucose and glutamate uptake, enhancing antioxidant capacity.
  • Peroxisomes play a critical role in fatty acid metabolism and redox control, interacting closely with mitochondria.

Clinical Implications

Clinicians should consider the metabolic adaptations in Barth syndrome when managing patients, particularly regarding oxidative stress and energy metabolism.

Conclusion

The study highlights the importance of peroxisomal function in mitigating oxidative stress in Barth syndrome.

Related Resources & Content

  1. Basic Research in Cardiology, 2023 -- Reprogramming of Cardiac Metabolism in Barth Syndrome Through Activation of the Integrated Stress Response
  2. Archives of Toxicology, 2015 -- Mitochondrial Reactive Oxygen Species and Their Influence on Glucose Metabolism in Cells
  3. Archives of Toxicology, 2025 -- The Impact of Redox Signaling on Mitochondrial and Endoplasmic Reticulum Function in Kidney Disorders
  4. Barth Syndrome - GeneReviews®, NCBI Bookshelf
  5. FDA Grants Accelerated Approval to First Treatment for Barth Syndrome | FDA
  6. Archives of Toxicology — Alterations in the Antioxidant System Induced by Metabolic Dysfunction-Associated Steatotic Liver Disease: A Comprehensive Review
  7. Pexophagy and Oxidative Stress: Focus on Peroxisomal Proteins and Reactive Oxygen Species (ROS) Signaling Pathways
  8. Barth Syndrome - GeneReviews® - NCBI Bookshelf
  9. FDA Grants Accelerated Approval to First Treatment for Barth Syndrome | FDA

Original Source(s)

Peroxisomal catalase and plasmalogen biosynthesis protect from oxidative stress in Barth syndrome cardiomyopathy

  • by Elsie Kajese, Malte Hachmann, Katharina J. Ermer, Manuela Erk, Lin Alhasaan, Michael Kohlhaas, Heike Bömmel, Lisa Berberich, Christopher Carlein, Hanna Eberl, Katrin Streckfuß-Bömeke, Leticia Prates Roma, Süleyman Ergün, Christoph Maack, Srikanth Karnati, Jan Dudek

  • June 30, 2026

Related Content

Risk factors of atrial fibrillation progression in patients with congenital heart disease

Application of predicting risk of cardiovascular disease events equations on postoperative major adverse cardiac and cerebral events for patients undergoing thoracic surgery

Relationship between persistent atrial fibrillation in high-altitude regions and left atrial diameter, red cell distribution width, and NT-ProBNP: a retrospective case-control study