Phytanic acid disturbs mitochondrial homeostasis in heart of young rats: a possible pathomechanism of cardiomyopathy in Refsum disease

Phytanic acid (Phyt) accumulates in tissues and biological fluids of patients affected by Refsum disease. Although cardiomyopathy is an important clinical manifestation of this disorder, the mechanisms of heart damage are poorly known. In the present study, we investigated the in vitro effects of Ph...

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Bibliographic Details
Published in:Molecular and cellular biochemistry 2012-07, Vol.366 (1-2), p.335-343
Main Authors: Grings, Mateus, Tonin, Anelise Miotti, Knebel, Lisiane Aurélio, Zanatta, Ângela, Moura, Alana Pimentel, Filho, Carlos Severo Dutra, Wajner, Moacir, Leipnitz, Guilhian
Format: Article
Language:English
Subjects:
Animals
Antioxidants - pharmacology
Biochemistry
Biomedical and Life Sciences
Carbonyl compounds
Cardiology
Cardiomyopathies - metabolism
Cardiomyopathy
Chromans - pharmacology
Cytochrome c
Electron Transport Chain Complex Proteins - metabolism
Fatty acids
Glutathione - pharmacology
Heart
Heart diseases
Homeostasis
Homeostasis - drug effects
In Vitro Techniques
Life Sciences
Male
Medical Biochemistry
Membrane Potential, Mitochondrial - drug effects
Mitochondria
Mitochondria, Heart - drug effects
Mitochondria, Heart - metabolism
Mitochondrial DNA
Myocardium - metabolism
Myocardium - pathology
NADP - metabolism
NG-Nitroarginine Methyl Ester - pharmacology
Nitric oxide
Nitric Oxide Synthase - antagonists & inhibitors
Nitrogen oxide
Oncology
Oxidation
Oxidation-Reduction
Oxidative Stress
Phytanic Acid - pharmacology
Protein Carbonylation
Rats
Rats, Wistar
Refsum Disease - metabolism
Rodents
Thiobarbituric Acid Reactive Substances - metabolism
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Summary:Phytanic acid (Phyt) accumulates in tissues and biological fluids of patients affected by Refsum disease. Although cardiomyopathy is an important clinical manifestation of this disorder, the mechanisms of heart damage are poorly known. In the present study, we investigated the in vitro effects of Phyt on important parameters of oxidative stress in heart of young rats. Phyt significantly increased thiobarbituric acid-reactive substances levels ( P  
ISSN:0300-8177
1573-4919
DOI:10.1007/s11010-012-1311-1