LC-MS Based Platform Simplifies Access to Metabolomics for Peroxisomal Disorders [PDF]
Metabolites, 2021 Peroxisomes are central hubs for cell metabolism and their dysfunction is linked to devastating human disorders, such as peroxisomal biogenesis disorders and single peroxisomal enzyme/protein deficiencies.
Henry Gerd Klemp +5 more
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Multivariate analysis and model building for classifying patients in the peroxisomal disorders X-linked adrenoleukodystrophy and Zellweger syndrome in Chinese pediatric patients [PDF]
Orphanet Journal of Rare Diseases, 2023 Background The peroxisome is a ubiquitous single membrane-enclosed organelle with an important metabolic role. Peroxisomal disorders represent a class of medical conditions caused by deficiencies in peroxisome function and are segmented into enzyme-and ...
Zhixing Zhu +7 more
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Pediatric Neurology Briefs, 1990 The total fatty acid and aldehyde composition in the brain, liver, and kidneys of two infants with Zellweger’s syndrome and one with pseudo-Zellweger’s syndrome and the fatty acid patterns expressed as percent values are reported from the Autonomous ...
J Gordon Millichap
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Modelling Peroxisomal Disorders in Zebrafish [PDF]
CellsPeroxisomes are ubiquitous, dynamic, oxidative organelles with key functions in cellular lipid metabolism and redox homeostasis. They have been linked to healthy ageing, neurodegeneration, cancer, the combat of pathogens and viruses, and infection and ...
Chenxing S. Jiang, Michael Schrader
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Detection and Quantification of Free Radicals in Peroxisomal Disorders: A Comparative Study with Oxidative Stress Parameters [PDF]
Journal of Clinical and Diagnostic Research, 2015 Introduction: Free radicals have been thought to participate in pathogenesis of peroxisomal disorders. Objective: The aim of the work is to detect free oxide radicals in blood of patients with peroxisomal disorders and to study their relation with ...
Sohair Abd-El Mawgood Abd-ElMaksoud +8 more
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Insights Into the Peroxisomal Protein Inventory of Zebrafish
Frontiers in Physiology, 2022 Peroxisomes are ubiquitous, oxidative subcellular organelles with important functions in cellular lipid metabolism and redox homeostasis. Loss of peroxisomal functions causes severe disorders with developmental and neurological abnormalities.
Maki Kamoshita +8 more
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Pediatric Neurology Briefs, 1988 Generalized peroxisomal disorders are classified in three main groups in a review article from the Kennedy Institute and the Departments of Neurology and Pediatrics, Johns Hopkins University, 707 N. Broadway, Baltimore, MD.
J Gordon Millichap
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Peroxisomes in intracellular cholesterol transport: from basic physiology to brain pathology
Exploration of Neuroprotective Therapy, 2021 Peroxisomes are actively involved in the metabolism of various lipids including fatty acids, ether phospholipids, bile acids as well as the processing of reactive oxygen and nitrogen species.
Jian Xiao, Bao-Liang Song, Jie Luo
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Edgetic Perturbations Contribute to Phenotypic Variability in PEX26 Deficiency
Frontiers in Genetics, 2021 Peroxisomes share metabolic pathways with other organelles and peroxisomes are embedded into key cellular processes. However, the specific function of many peroxisomal proteins remains unclear and restricted knowledge of the peroxisomal protein ...
Amelie S. Lotz-Havla +7 more
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MoPex19, which is essential for maintenance of peroxisomal structure and woronin bodies, is required for metabolism and development in the rice blast fungus. [PDF]
PLoS ONE, 2014 Peroxisomes are present ubiquitously and make important contributions to cellular metabolism in eukaryotes. They play crucial roles in pathogenicity of plant fungal pathogens.
Ling Li +10 more
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