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Mitochondria are eukaryotic cellular organelles that are known to produce energy through a process called oxidative phosphorylation. Besides their primary function, mitochondria are involved in various cellular processes, including cell growth, differentiation, signaling, metabolism, and senescence. Age-related changes cause a decline in mitochondrial quality and integrity due to increased mitochondrial mutations and oxidative damage. Thus, aging can severely impact mitochondrial functions,...
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The mitochondrial electron transport chain (ETC) is the main energy generation system in the eukaryotic cells. However, mitochondria also produce cytotoxic reactive oxygen species (ROS) due to the large electron flow during oxidative phosphorylation. While Complex I is one of the primary sources of superoxide radicals, ROS production by Complex II is uncommon and may only be observed in cancer cells with mutated complexes.
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A single mitochondrion is a bean-shaped organelle enclosed by a double-membrane system. The outer membrane of mitochondria is smooth and contains many porins - the integral membrane transporters. Porins enable free diffusion of ions and small uncharged molecules through the outer mitochondrial membrane but limit the transport of molecules larger than 5000 Daltons. Further, the outer mitochondrial membrane forms a unique structure called membrane contact sites with other subcellular organelles,...
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  11. Role Of Mitochondrial Function In The Oxidative Stress Profile Of Children With Prader-willi Syndrome

Role of mitochondrial function in the oxidative stress profile of children with Prader-Willi syndrome

Álvaro Carrasco-García1, Guadalupe Herrera2, Laura C G de Graaff3

  • 1Department of Internal Medicine, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands; Department of Pediatrics, Obstetrics and Gynecology, University of Valencia, Valencia, Spain.

Free Radical Biology & Medicine
|June 13, 2025

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View abstract on PubMed

Summary
This summary is machine-generated.

Growth hormone (GH) treatment in Prader-Willi syndrome (PWS) children impacts oxidative stress and inflammation. Despite lower glutathione, GH-treated PWS patients show enhanced mitochondrial function and antioxidant capacity compared to obese controls.

Area of Science:

  • Endocrinology
  • Genetics
  • Cell Biology

Background:

  • Prader-Willi syndrome (PWS) is a rare genetic disorder linked to severe obesity and increased oxidative stress.
  • Oxidative stress, driven by reactive oxygen species (ROS), contributes to inflammation and metabolic dysfunction in PWS.
  • Growth hormone (GH) therapy is standard for PWS, improving body composition, motor, and cognitive functions.

Purpose of the Study:

  • To investigate the oxidative stress profile in GH-treated children with PWS.
  • To compare oxidative stress markers between GH-treated PWS children and non-syndromic obese children.
  • To explore the role of mitochondrial function and inflammation in PWS under GH treatment.

Main Methods:

  • Flow cytometry was used to analyze markers of oxidative stress, inflammation, and mitochondrial function.
Keywords:
Prader-Willi syndromeageingmitochondrial functionoxidative stress

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  • Immune cell populations related to inflammation and immune response were identified.
  • 12 GH-treated PWS children and 11 non-syndromic obese children were included.

    • Main Results:

    • GH-treated PWS children had significantly lower glutathione (GSH) levels but 2- to 3.5-fold higher mitochondrial activity.
    • PWS patients exhibited reduced lipid peroxidation and protein carbonylation, indicating increased antioxidant capacity.
    • Despite being non-obese, GH-treated PWS children showed higher systemic inflammation and elevated inflammatory biomarkers compared to obese controls.

    Conclusions:

    • Mitochondrial pathways appear crucial for antioxidant responses in PWS.
    • GH treatment may modulate oxidative stress and inflammation in PWS, potentially influencing senescence and premature aging.
    • Further research into these mechanisms can guide targeted therapies for PWS management.