Loading…
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...
Saved in:
| Published in: | Molecular and cellular biochemistry 2012-07, Vol.366 (1-2), p.335-343 |
|---|---|
| Main Authors: | , , , , , , , |
| Format: | Article |
| Language: | English |
| Subjects: | |
| Citations: | Items that this one cites Items that cite this one |
| Online Access: | Get full text |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| cited_by | cdi_FETCH-LOGICAL-c439t-cd8aadac78a6c777b0919225e14d22e063ccb768d8f42e43c34da1cb937e7c333 |
|---|---|
| cites | cdi_FETCH-LOGICAL-c439t-cd8aadac78a6c777b0919225e14d22e063ccb768d8f42e43c34da1cb937e7c333 |
| container_end_page | 343 |
| container_issue | 1-2 |
| container_start_page | 335 |
| container_title | Molecular and cellular biochemistry |
| container_volume | 366 |
| creator | Grings, Mateus Tonin, Anelise Miotti Knebel, Lisiane Aurélio Zanatta, Ângela Moura, Alana Pimentel Filho, Carlos Severo Dutra Wajner, Moacir Leipnitz, Guilhian |
| description | 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
|
| doi_str_mv | 10.1007/s11010-012-1311-1 |
| format | article |
| fullrecord | <record><control><sourceid>gale_proqu</sourceid><recordid>TN_cdi_proquest_miscellaneous_1019094509</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><galeid>A359411107</galeid><sourcerecordid>A359411107</sourcerecordid><originalsourceid>FETCH-LOGICAL-c439t-cd8aadac78a6c777b0919225e14d22e063ccb768d8f42e43c34da1cb937e7c333</originalsourceid><addsrcrecordid>eNp1kduK1TAUhoMoznbrA3gjAW-86ZjVtDuNd8PgCQYU0euymqzuZmibbZJe9AV8blP2eEQJJJD1rX8dfsaegrgEIdTLCCBAFALKAiRAAffYDmoli0qDvs92QgpRNKDUBXsU463IsAB4yC7Ksi6Vls2Offs4rAlnZzgaZ7l1MS2hi3xyyZvBzzY4HPngJ_IxYXSRu5kPhCFx3_PVL_ORB0zxFUd-8jG6biR-wrRlmCELx2kDDQbr_LT6LbRuGp-oj8u0FSSM9Jg96HGM9OTu3bMvb15_vn5X3Hx4-_766qYwldSpMLZBtGhUgwejlOpEHjQPQ1DZsiRxkMZ06tDYpq9KqqSRlUUwnZaKlJFS7tmLs-4p-K8LxdROLhoaR5zJL7HN-9RCV7XQGX3-F3rrlzDn7jaqOZQaavGLOuJIrZt7nwKaTbS9krWuIFukMnX5DyofS5Mzfqbe5f8_EuCcYEJeaqC-PQU3YVhz7Xbzvj1732bv2837fO3Zs7uGl24i-zPjh9kZKM9AzKH5SOH3if6n-h1YErnK</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>1018629150</pqid></control><display><type>article</type><title>Phytanic acid disturbs mitochondrial homeostasis in heart of young rats: a possible pathomechanism of cardiomyopathy in Refsum disease</title><source>Springer Link</source><creator>Grings, Mateus ; Tonin, Anelise Miotti ; Knebel, Lisiane Aurélio ; Zanatta, Ângela ; Moura, Alana Pimentel ; Filho, Carlos Severo Dutra ; Wajner, Moacir ; Leipnitz, Guilhian</creator><creatorcontrib>Grings, Mateus ; Tonin, Anelise Miotti ; Knebel, Lisiane Aurélio ; Zanatta, Ângela ; Moura, Alana Pimentel ; Filho, Carlos Severo Dutra ; Wajner, Moacir ; Leipnitz, Guilhian</creatorcontrib><description>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
< 0.001) and carbonyl formation (
P
< 0.01), indicating that this fatty acid induces lipid and protein oxidative damage, respectively. In contrast, Phyt did not alter sulfhydryl oxidation. Phyt also decreased glutathione (GSH) concentrations (
P
< 0.05), an important non-enzymatic antioxidant defense. Moreover, Phyt increased 2′,7′-dichlorofluorescin oxidation (DCFH) (
P
< 0.01), reflecting increased reactive species generation. We also found that the induced lipid and protein oxidative damage, as well as the decreased GSH levels and increased DCFH oxidation provoked by this fatty acid were prevented or attenuated by the reactive oxygen species scavengers melatonin, trolox, and GSH, but not by the nitric oxide inhibitor
n
ω
-nitro-
l
-arginine methyl ester, suggesting that reactive oxygen species were involved in these effects. Next, we verified that Phyt strongly inhibited NADH-cytochrome c oxidoreductase (complex I–III) activity (
P
< 0.001) in heart supernatants, and decreased membrane potential and the NAD(P)H pool in heart mitochondria, indicating that Phyt acts as a metabolic inhibitor and as an uncoupler of the electron transport chain. Therefore, it can be presumed that disturbance of cellular energy and redox homeostasis induced by Phyt may possibly contribute to the cardiomyopathy found in patients affected by Refsum disease.</description><identifier>ISSN: 0300-8177</identifier><identifier>EISSN: 1573-4919</identifier><identifier>DOI: 10.1007/s11010-012-1311-1</identifier><identifier>PMID: 22527938</identifier><language>eng</language><publisher>Boston: Springer US</publisher><subject>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</subject><ispartof>Molecular and cellular biochemistry, 2012-07, Vol.366 (1-2), p.335-343</ispartof><rights>Springer Science+Business Media, LLC. 2012</rights><rights>COPYRIGHT 2012 Springer</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c439t-cd8aadac78a6c777b0919225e14d22e063ccb768d8f42e43c34da1cb937e7c333</citedby><cites>FETCH-LOGICAL-c439t-cd8aadac78a6c777b0919225e14d22e063ccb768d8f42e43c34da1cb937e7c333</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/22527938$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Grings, Mateus</creatorcontrib><creatorcontrib>Tonin, Anelise Miotti</creatorcontrib><creatorcontrib>Knebel, Lisiane Aurélio</creatorcontrib><creatorcontrib>Zanatta, Ângela</creatorcontrib><creatorcontrib>Moura, Alana Pimentel</creatorcontrib><creatorcontrib>Filho, Carlos Severo Dutra</creatorcontrib><creatorcontrib>Wajner, Moacir</creatorcontrib><creatorcontrib>Leipnitz, Guilhian</creatorcontrib><title>Phytanic acid disturbs mitochondrial homeostasis in heart of young rats: a possible pathomechanism of cardiomyopathy in Refsum disease</title><title>Molecular and cellular biochemistry</title><addtitle>Mol Cell Biochem</addtitle><addtitle>Mol Cell Biochem</addtitle><description>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
< 0.001) and carbonyl formation (
P
< 0.01), indicating that this fatty acid induces lipid and protein oxidative damage, respectively. In contrast, Phyt did not alter sulfhydryl oxidation. Phyt also decreased glutathione (GSH) concentrations (
P
< 0.05), an important non-enzymatic antioxidant defense. Moreover, Phyt increased 2′,7′-dichlorofluorescin oxidation (DCFH) (
P
< 0.01), reflecting increased reactive species generation. We also found that the induced lipid and protein oxidative damage, as well as the decreased GSH levels and increased DCFH oxidation provoked by this fatty acid were prevented or attenuated by the reactive oxygen species scavengers melatonin, trolox, and GSH, but not by the nitric oxide inhibitor
n
ω
-nitro-
l
-arginine methyl ester, suggesting that reactive oxygen species were involved in these effects. Next, we verified that Phyt strongly inhibited NADH-cytochrome c oxidoreductase (complex I–III) activity (
P
< 0.001) in heart supernatants, and decreased membrane potential and the NAD(P)H pool in heart mitochondria, indicating that Phyt acts as a metabolic inhibitor and as an uncoupler of the electron transport chain. Therefore, it can be presumed that disturbance of cellular energy and redox homeostasis induced by Phyt may possibly contribute to the cardiomyopathy found in patients affected by Refsum disease.</description><subject>Animals</subject><subject>Antioxidants - pharmacology</subject><subject>Biochemistry</subject><subject>Biomedical and Life Sciences</subject><subject>Carbonyl compounds</subject><subject>Cardiology</subject><subject>Cardiomyopathies - metabolism</subject><subject>Cardiomyopathy</subject><subject>Chromans - pharmacology</subject><subject>Cytochrome c</subject><subject>Electron Transport Chain Complex Proteins - metabolism</subject><subject>Fatty acids</subject><subject>Glutathione - pharmacology</subject><subject>Heart</subject><subject>Heart diseases</subject><subject>Homeostasis</subject><subject>Homeostasis - drug effects</subject><subject>In Vitro Techniques</subject><subject>Life Sciences</subject><subject>Male</subject><subject>Medical Biochemistry</subject><subject>Membrane Potential, Mitochondrial - drug effects</subject><subject>Mitochondria</subject><subject>Mitochondria, Heart - drug effects</subject><subject>Mitochondria, Heart - metabolism</subject><subject>Mitochondrial DNA</subject><subject>Myocardium - metabolism</subject><subject>Myocardium - pathology</subject><subject>NADP - metabolism</subject><subject>NG-Nitroarginine Methyl Ester - pharmacology</subject><subject>Nitric oxide</subject><subject>Nitric Oxide Synthase - antagonists & inhibitors</subject><subject>Nitrogen oxide</subject><subject>Oncology</subject><subject>Oxidation</subject><subject>Oxidation-Reduction</subject><subject>Oxidative Stress</subject><subject>Phytanic Acid - pharmacology</subject><subject>Protein Carbonylation</subject><subject>Rats</subject><subject>Rats, Wistar</subject><subject>Refsum Disease - metabolism</subject><subject>Rodents</subject><subject>Thiobarbituric Acid Reactive Substances - metabolism</subject><issn>0300-8177</issn><issn>1573-4919</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2012</creationdate><recordtype>article</recordtype><recordid>eNp1kduK1TAUhoMoznbrA3gjAW-86ZjVtDuNd8PgCQYU0euymqzuZmibbZJe9AV8blP2eEQJJJD1rX8dfsaegrgEIdTLCCBAFALKAiRAAffYDmoli0qDvs92QgpRNKDUBXsU463IsAB4yC7Ksi6Vls2Offs4rAlnZzgaZ7l1MS2hi3xyyZvBzzY4HPngJ_IxYXSRu5kPhCFx3_PVL_ORB0zxFUd-8jG6biR-wrRlmCELx2kDDQbr_LT6LbRuGp-oj8u0FSSM9Jg96HGM9OTu3bMvb15_vn5X3Hx4-_766qYwldSpMLZBtGhUgwejlOpEHjQPQ1DZsiRxkMZ06tDYpq9KqqSRlUUwnZaKlJFS7tmLs-4p-K8LxdROLhoaR5zJL7HN-9RCV7XQGX3-F3rrlzDn7jaqOZQaavGLOuJIrZt7nwKaTbS9krWuIFukMnX5DyofS5Mzfqbe5f8_EuCcYEJeaqC-PQU3YVhz7Xbzvj1732bv2837fO3Zs7uGl24i-zPjh9kZKM9AzKH5SOH3if6n-h1YErnK</recordid><startdate>20120701</startdate><enddate>20120701</enddate><creator>Grings, Mateus</creator><creator>Tonin, Anelise Miotti</creator><creator>Knebel, Lisiane Aurélio</creator><creator>Zanatta, Ângela</creator><creator>Moura, Alana Pimentel</creator><creator>Filho, Carlos Severo Dutra</creator><creator>Wajner, Moacir</creator><creator>Leipnitz, Guilhian</creator><general>Springer US</general><general>Springer</general><general>Springer Nature B.V</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7QL</scope><scope>7QP</scope><scope>7T5</scope><scope>7T7</scope><scope>7TK</scope><scope>7TM</scope><scope>7TO</scope><scope>7U9</scope><scope>7X7</scope><scope>7XB</scope><scope>88A</scope><scope>88E</scope><scope>88I</scope><scope>8AO</scope><scope>8FD</scope><scope>8FE</scope><scope>8FH</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BBNVY</scope><scope>BENPR</scope><scope>BHPHI</scope><scope>C1K</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FR3</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>H94</scope><scope>HCIFZ</scope><scope>K9.</scope><scope>LK8</scope><scope>M0S</scope><scope>M1P</scope><scope>M2P</scope><scope>M7N</scope><scope>M7P</scope><scope>P64</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>Q9U</scope><scope>RC3</scope><scope>7X8</scope></search><sort><creationdate>20120701</creationdate><title>Phytanic acid disturbs mitochondrial homeostasis in heart of young rats: a possible pathomechanism of cardiomyopathy in Refsum disease</title><author>Grings, Mateus ; Tonin, Anelise Miotti ; Knebel, Lisiane Aurélio ; Zanatta, Ângela ; Moura, Alana Pimentel ; Filho, Carlos Severo Dutra ; Wajner, Moacir ; Leipnitz, Guilhian</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c439t-cd8aadac78a6c777b0919225e14d22e063ccb768d8f42e43c34da1cb937e7c333</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2012</creationdate><topic>Animals</topic><topic>Antioxidants - pharmacology</topic><topic>Biochemistry</topic><topic>Biomedical and Life Sciences</topic><topic>Carbonyl compounds</topic><topic>Cardiology</topic><topic>Cardiomyopathies - metabolism</topic><topic>Cardiomyopathy</topic><topic>Chromans - pharmacology</topic><topic>Cytochrome c</topic><topic>Electron Transport Chain Complex Proteins - metabolism</topic><topic>Fatty acids</topic><topic>Glutathione - pharmacology</topic><topic>Heart</topic><topic>Heart diseases</topic><topic>Homeostasis</topic><topic>Homeostasis - drug effects</topic><topic>In Vitro Techniques</topic><topic>Life Sciences</topic><topic>Male</topic><topic>Medical Biochemistry</topic><topic>Membrane Potential, Mitochondrial - drug effects</topic><topic>Mitochondria</topic><topic>Mitochondria, Heart - drug effects</topic><topic>Mitochondria, Heart - metabolism</topic><topic>Mitochondrial DNA</topic><topic>Myocardium - metabolism</topic><topic>Myocardium - pathology</topic><topic>NADP - metabolism</topic><topic>NG-Nitroarginine Methyl Ester - pharmacology</topic><topic>Nitric oxide</topic><topic>Nitric Oxide Synthase - antagonists & inhibitors</topic><topic>Nitrogen oxide</topic><topic>Oncology</topic><topic>Oxidation</topic><topic>Oxidation-Reduction</topic><topic>Oxidative Stress</topic><topic>Phytanic Acid - pharmacology</topic><topic>Protein Carbonylation</topic><topic>Rats</topic><topic>Rats, Wistar</topic><topic>Refsum Disease - metabolism</topic><topic>Rodents</topic><topic>Thiobarbituric Acid Reactive Substances - metabolism</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Grings, Mateus</creatorcontrib><creatorcontrib>Tonin, Anelise Miotti</creatorcontrib><creatorcontrib>Knebel, Lisiane Aurélio</creatorcontrib><creatorcontrib>Zanatta, Ângela</creatorcontrib><creatorcontrib>Moura, Alana Pimentel</creatorcontrib><creatorcontrib>Filho, Carlos Severo Dutra</creatorcontrib><creatorcontrib>Wajner, Moacir</creatorcontrib><creatorcontrib>Leipnitz, Guilhian</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Bacteriology Abstracts (Microbiology B)</collection><collection>Calcium & Calcified Tissue Abstracts</collection><collection>Immunology Abstracts</collection><collection>Industrial and Applied Microbiology Abstracts (Microbiology A)</collection><collection>Neurosciences Abstracts</collection><collection>Nucleic Acids Abstracts</collection><collection>Oncogenes and Growth Factors Abstracts</collection><collection>Virology and AIDS Abstracts</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Biology Database (Alumni Edition)</collection><collection>Medical Database (Alumni Edition)</collection><collection>Science Database (Alumni Edition)</collection><collection>ProQuest Pharma Collection</collection><collection>Technology Research Database</collection><collection>ProQuest SciTech Collection</collection><collection>ProQuest Natural Science Collection</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni)</collection><collection>ProQuest Central</collection><collection>ProQuest Central Essentials</collection><collection>Biological Science Collection</collection><collection>ProQuest Central</collection><collection>ProQuest Natural Science Collection</collection><collection>Environmental Sciences and Pollution Management</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central</collection><collection>Engineering Research Database</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Central Student</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>SciTech Premium Collection</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Biological Sciences</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Science Database</collection><collection>Algology Mycology and Protozoology Abstracts (Microbiology C)</collection><collection>ProQuest Biological Science Journals</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central Basic</collection><collection>Genetics Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>Molecular and cellular biochemistry</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Grings, Mateus</au><au>Tonin, Anelise Miotti</au><au>Knebel, Lisiane Aurélio</au><au>Zanatta, Ângela</au><au>Moura, Alana Pimentel</au><au>Filho, Carlos Severo Dutra</au><au>Wajner, Moacir</au><au>Leipnitz, Guilhian</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Phytanic acid disturbs mitochondrial homeostasis in heart of young rats: a possible pathomechanism of cardiomyopathy in Refsum disease</atitle><jtitle>Molecular and cellular biochemistry</jtitle><stitle>Mol Cell Biochem</stitle><addtitle>Mol Cell Biochem</addtitle><date>2012-07-01</date><risdate>2012</risdate><volume>366</volume><issue>1-2</issue><spage>335</spage><epage>343</epage><pages>335-343</pages><issn>0300-8177</issn><eissn>1573-4919</eissn><abstract>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
< 0.001) and carbonyl formation (
P
< 0.01), indicating that this fatty acid induces lipid and protein oxidative damage, respectively. In contrast, Phyt did not alter sulfhydryl oxidation. Phyt also decreased glutathione (GSH) concentrations (
P
< 0.05), an important non-enzymatic antioxidant defense. Moreover, Phyt increased 2′,7′-dichlorofluorescin oxidation (DCFH) (
P
< 0.01), reflecting increased reactive species generation. We also found that the induced lipid and protein oxidative damage, as well as the decreased GSH levels and increased DCFH oxidation provoked by this fatty acid were prevented or attenuated by the reactive oxygen species scavengers melatonin, trolox, and GSH, but not by the nitric oxide inhibitor
n
ω
-nitro-
l
-arginine methyl ester, suggesting that reactive oxygen species were involved in these effects. Next, we verified that Phyt strongly inhibited NADH-cytochrome c oxidoreductase (complex I–III) activity (
P
< 0.001) in heart supernatants, and decreased membrane potential and the NAD(P)H pool in heart mitochondria, indicating that Phyt acts as a metabolic inhibitor and as an uncoupler of the electron transport chain. Therefore, it can be presumed that disturbance of cellular energy and redox homeostasis induced by Phyt may possibly contribute to the cardiomyopathy found in patients affected by Refsum disease.</abstract><cop>Boston</cop><pub>Springer US</pub><pmid>22527938</pmid><doi>10.1007/s11010-012-1311-1</doi><tpages>9</tpages></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0300-8177 |
| ispartof | Molecular and cellular biochemistry, 2012-07, Vol.366 (1-2), p.335-343 |
| issn | 0300-8177 1573-4919 |
| language | eng |
| recordid | cdi_proquest_miscellaneous_1019094509 |
| source | Springer Link |
| 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 |
| title | Phytanic acid disturbs mitochondrial homeostasis in heart of young rats: a possible pathomechanism of cardiomyopathy in Refsum disease |
| url | http://sfxeu10.hosted.exlibrisgroup.com/loughborough?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-01T14%3A20%3A32IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-gale_proqu&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Phytanic%20acid%20disturbs%20mitochondrial%20homeostasis%20in%20heart%20of%20young%20rats:%20a%20possible%20pathomechanism%20of%20cardiomyopathy%20in%20Refsum%20disease&rft.jtitle=Molecular%20and%20cellular%20biochemistry&rft.au=Grings,%20Mateus&rft.date=2012-07-01&rft.volume=366&rft.issue=1-2&rft.spage=335&rft.epage=343&rft.pages=335-343&rft.issn=0300-8177&rft.eissn=1573-4919&rft_id=info:doi/10.1007/s11010-012-1311-1&rft_dat=%3Cgale_proqu%3EA359411107%3C/gale_proqu%3E%3Cgrp_id%3Ecdi_FETCH-LOGICAL-c439t-cd8aadac78a6c777b0919225e14d22e063ccb768d8f42e43c34da1cb937e7c333%3C/grp_id%3E%3Coa%3E%3C/oa%3E%3Curl%3E%3C/url%3E&rft_id=info:oai/&rft_pqid=1018629150&rft_id=info:pmid/22527938&rft_galeid=A359411107&rfr_iscdi=true |