Peroxisomes and their role in pediatric diseases

Peroxisomes are specialized single-membrane organelles that play a key role in lipid metabolism, detoxification, and redox regulation. Disruptions in their biogenesis and function lead to severe inherited diseases, including Zellweger spectrum disorders and rhizomelic chondrodysplasia punctata. Despite significant advances in peroxisomal research, their involvement in various pathologies, including viral infections and immune disorders, remains insufficiently studied. Objective: To analyze current data on the structure, functions, and interactions of peroxisomes with other organelles, their role in human metabolism, immune processes, and the pathogenesis of diseases associated with peroxisomal protein defects. Key points. Peroxisomes participate in fatty acid oxidation, ether lipid biosynthesis, and reactive oxygen species metabolism. Their interactions with mitochondria, lysosomes, and the endoplasmic reticulum play a crucial role in cellular homeostasis. Peroxisomal metabolic dysfunction is linked not only to genetic diseases but also to altered immune responses, viral infections, and neurodegenerative processes. This review presents contemporary insights into the mechanisms underlying peroxisomal disorders and potential therapeutic approaches. Conclusion. Peroxisomal defects represent complex systemic disorders requiring a multidisciplinary approach. Modern molecular technologies enable the identification of novel disease mechanisms and the development of personalized treatment strategies. Further research is needed to clarify their role in immune regulation and infectious pathology.

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Publication Details

Journal
Russian Journal of Evidence-Based Gastroenterology
Published
2025-03-27
DOI
https://doi.org/10.17116/dokgastro20251401191
Primary Topic
Peroxisome Proliferator-Activated Receptors
Type
article
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article

Peroxisomes and their role in pediatric diseases

Г. В. Волынец, А. В. Никитин, А. И. Хавкин, Т. А. Скворцова
Russian Journal of Evidence-Based Gastroenterology
Peroxisome Proliferator-Activated Receptors
article

Peroxisomes and their role in pediatric diseases

Г. В. Волынец, А. В. Никитин, А. И. Хавкин, Т. А. Скворцова
article en

Abstract

Peroxisomes are specialized single-membrane organelles that play a key role in lipid metabolism, detoxification, and redox regulation. Disruptions in their biogenesis and function lead to severe inherited diseases, including Zellweger spectrum disorders and rhizomelic chondrodysplasia punctata. Despite significant advances in peroxisomal research, their involvement in various pathologies, including viral infections and immune disorders, remains insufficiently studied. Objective: To analyze current data on the structure, functions, and interactions of peroxisomes with other organelles, their role in human metabolism, immune processes, and the pathogenesis of diseases associated with peroxisomal protein defects. Key points. Peroxisomes participate in fatty acid oxidation, ether lipid biosynthesis, and reactive oxygen species metabolism. Their interactions with mitochondria, lysosomes, and the endoplasmic reticulum play a crucial role in cellular homeostasis. Peroxisomal metabolic dysfunction is linked not only to genetic diseases but also to altered immune responses, viral infections, and neurodegenerative processes. This review presents contemporary insights into the mechanisms underlying peroxisomal disorders and potential therapeutic approaches. Conclusion. Peroxisomal defects represent complex systemic disorders requiring a multidisciplinary approach. Modern molecular technologies enable the identification of novel disease mechanisms and the development of personalized treatment strategies. Further research is needed to clarify their role in immune regulation and infectious pathology.

Russian Journal of Evidence-Based GastroenterologyVol. 14(1)
Pirogov Russian National Research Medical University (RU), Russian Children's Clinical Hospital (RU), City Clinical Hospital (RU)
Openalex Percentile: Top 95%
Peroxisome Proliferator-Activated Receptors
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