Loading…
Intermediates of unsaturated fatty acid oxidation are incorporated in triglycerides but not in phospholipids in tissues from patients with mitochondrial β‐oxidation defects
The fatty acid composition was determined of liver, skeletal muscle and heart obtained post mortem from patients with medium‐chain acyl‐CoA dehydrogenase deficiency (MCADD), multiple acyl‐CoA dehydrogenase deficiency (MADD) and very long‐chain acyl‐CoA dehydrogenase deficiency (VLCADD). Increased am...
Saved in:
Published in: | Journal of inherited metabolic disease 2001-06, Vol.24 (3), p.337-344 |
---|---|
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-c3697-c873c758995c04946f2a2c3ebc18da2a0bf0167e894d927344acdec83f0d56be3 |
---|---|
cites | cdi_FETCH-LOGICAL-c3697-c873c758995c04946f2a2c3ebc18da2a0bf0167e894d927344acdec83f0d56be3 |
container_end_page | 344 |
container_issue | 3 |
container_start_page | 337 |
container_title | Journal of inherited metabolic disease |
container_volume | 24 |
creator | Onkenhout, W. Venizelos, V. Scholte, H. R. Klerk, J. B. C. Poorthuis, B. J. H. M. |
description | The fatty acid composition was determined of liver, skeletal muscle and heart obtained post mortem from patients with medium‐chain acyl‐CoA dehydrogenase deficiency (MCADD), multiple acyl‐CoA dehydrogenase deficiency (MADD) and very long‐chain acyl‐CoA dehydrogenase deficiency (VLCADD). Increased amounts of 4‐decenoic acid 10:1(n−6), 5‐dodecenoic acid 12:1(n−7), 5‐tetradecenoic acid 14:1(n−9), 5,8‐tetradecadienoic acid 14:2(n−6) and 7,10‐hexadecadienoic acid 16:2(n−6)—intermediates of unsaturated fatty acid oxidation—were found. Fractionation into different lipid classes showed that these fatty acids were exclusively present in the triglyceride fraction. They could not be detected in the free fatty acid fraction or in the phospholipid fraction. Our results suggest that intermediates of unsaturated fatty acid oxidation that accumulate as a consequence of MCADD, MADD and VLCADD are transported to the endoplasmic reticulum for esterification into neutral glycerolipids. The pattern of accumulation is characteristic for each disease, which makes fatty acid analysis of total lipid of post‐mortem tissues a useful tool in the detection of mitochondrial fatty acid oxidation defects in patients who died unexpected, for example with sudden infant death syndrome. |
doi_str_mv | 10.1023/A:1010592232317 |
format | article |
fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_71059516</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>71059516</sourcerecordid><originalsourceid>FETCH-LOGICAL-c3697-c873c758995c04946f2a2c3ebc18da2a0bf0167e894d927344acdec83f0d56be3</originalsourceid><addsrcrecordid>eNqF0U2OFCEUB3BiNE47unZn2OiuHD6rwF1nHLXNGDe6rlB82JgqKIHKTO_mCN7ExIN4CE8ibXfSceWCEMKP_yPvAfAUo5cYEXqxfoURRlwSQgnF3T2wwryjDWlbfh-sEGa4EZLzM_Ao568IISk4fwjOMGaiFVKswI9NKDZN1nhVbIbRwSVkVZZUjwY6VcoOKu0NjLfeqOJjgCpZ6IOOaY4H5QMsyX8Zd9omb2rKsBQYYtlfzNuY6xr97E3-K33OSzUuxQnONdGGkuGNL1s4-RL1NgaTvBrhr5-_776fqhrrrC75MXjg1Jjtk-N-Dj6_ufp0-a65_vh2c7m-bjRtZddo0VHdcSEl14hJ1jqiiKZ20FgYRRQaHMJtZ4VkRpKOMqa0sVpQhwxvB0vPwYtD7pzit_rf0k8-azuOKti45L7bd53jtsKLA9Qp5pys6-fkJ5V2PUb9fkb9uv9nRvXFs2P0MtTGn_xxKBU8PwKVtRpdUkH7fHIMSc44q44f3I0f7e5_dfv3mw-vEaUd_QM6uK-1</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>71059516</pqid></control><display><type>article</type><title>Intermediates of unsaturated fatty acid oxidation are incorporated in triglycerides but not in phospholipids in tissues from patients with mitochondrial β‐oxidation defects</title><source>Springer Journals</source><source>Wiley-Blackwell Read & Publish Collection</source><creator>Onkenhout, W. ; Venizelos, V. ; Scholte, H. R. ; Klerk, J. B. C. ; Poorthuis, B. J. H. M.</creator><creatorcontrib>Onkenhout, W. ; Venizelos, V. ; Scholte, H. R. ; Klerk, J. B. C. ; Poorthuis, B. J. H. M.</creatorcontrib><description>The fatty acid composition was determined of liver, skeletal muscle and heart obtained post mortem from patients with medium‐chain acyl‐CoA dehydrogenase deficiency (MCADD), multiple acyl‐CoA dehydrogenase deficiency (MADD) and very long‐chain acyl‐CoA dehydrogenase deficiency (VLCADD). Increased amounts of 4‐decenoic acid 10:1(n−6), 5‐dodecenoic acid 12:1(n−7), 5‐tetradecenoic acid 14:1(n−9), 5,8‐tetradecadienoic acid 14:2(n−6) and 7,10‐hexadecadienoic acid 16:2(n−6)—intermediates of unsaturated fatty acid oxidation—were found. Fractionation into different lipid classes showed that these fatty acids were exclusively present in the triglyceride fraction. They could not be detected in the free fatty acid fraction or in the phospholipid fraction. Our results suggest that intermediates of unsaturated fatty acid oxidation that accumulate as a consequence of MCADD, MADD and VLCADD are transported to the endoplasmic reticulum for esterification into neutral glycerolipids. The pattern of accumulation is characteristic for each disease, which makes fatty acid analysis of total lipid of post‐mortem tissues a useful tool in the detection of mitochondrial fatty acid oxidation defects in patients who died unexpected, for example with sudden infant death syndrome.</description><identifier>ISSN: 0141-8955</identifier><identifier>EISSN: 1573-2665</identifier><identifier>DOI: 10.1023/A:1010592232317</identifier><identifier>PMID: 11486898</identifier><identifier>CODEN: JIMDDP</identifier><language>eng</language><publisher>Dordrecht: Kluwer Academic Publishers</publisher><subject>Acyl-CoA Dehydrogenase, Long-Chain - deficiency ; Biological and medical sciences ; Endoplasmic Reticulum - metabolism ; Errors of metabolism ; Esterification ; Fatal Outcome ; Fatty Acids, Unsaturated - analysis ; Fatty Acids, Unsaturated - metabolism ; Female ; Humans ; Infant ; Infant, Newborn ; Lipids (lysosomal enzyme disorders, storage diseases) ; Liver - chemistry ; Liver - metabolism ; Male ; Medical sciences ; Metabolic diseases ; Mitochondrial Diseases - metabolism ; Muscle, Skeletal - chemistry ; Muscle, Skeletal - metabolism ; Myocardium - chemistry ; Myocardium - metabolism ; Oxidation-Reduction ; Phospholipids - metabolism ; Triglycerides - metabolism</subject><ispartof>Journal of inherited metabolic disease, 2001-06, Vol.24 (3), p.337-344</ispartof><rights>2001 SSIEM</rights><rights>2002 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3697-c873c758995c04946f2a2c3ebc18da2a0bf0167e894d927344acdec83f0d56be3</citedby><cites>FETCH-LOGICAL-c3697-c873c758995c04946f2a2c3ebc18da2a0bf0167e894d927344acdec83f0d56be3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=14095454$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/11486898$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Onkenhout, W.</creatorcontrib><creatorcontrib>Venizelos, V.</creatorcontrib><creatorcontrib>Scholte, H. R.</creatorcontrib><creatorcontrib>Klerk, J. B. C.</creatorcontrib><creatorcontrib>Poorthuis, B. J. H. M.</creatorcontrib><title>Intermediates of unsaturated fatty acid oxidation are incorporated in triglycerides but not in phospholipids in tissues from patients with mitochondrial β‐oxidation defects</title><title>Journal of inherited metabolic disease</title><addtitle>J Inherit Metab Dis</addtitle><description>The fatty acid composition was determined of liver, skeletal muscle and heart obtained post mortem from patients with medium‐chain acyl‐CoA dehydrogenase deficiency (MCADD), multiple acyl‐CoA dehydrogenase deficiency (MADD) and very long‐chain acyl‐CoA dehydrogenase deficiency (VLCADD). Increased amounts of 4‐decenoic acid 10:1(n−6), 5‐dodecenoic acid 12:1(n−7), 5‐tetradecenoic acid 14:1(n−9), 5,8‐tetradecadienoic acid 14:2(n−6) and 7,10‐hexadecadienoic acid 16:2(n−6)—intermediates of unsaturated fatty acid oxidation—were found. Fractionation into different lipid classes showed that these fatty acids were exclusively present in the triglyceride fraction. They could not be detected in the free fatty acid fraction or in the phospholipid fraction. Our results suggest that intermediates of unsaturated fatty acid oxidation that accumulate as a consequence of MCADD, MADD and VLCADD are transported to the endoplasmic reticulum for esterification into neutral glycerolipids. The pattern of accumulation is characteristic for each disease, which makes fatty acid analysis of total lipid of post‐mortem tissues a useful tool in the detection of mitochondrial fatty acid oxidation defects in patients who died unexpected, for example with sudden infant death syndrome.</description><subject>Acyl-CoA Dehydrogenase, Long-Chain - deficiency</subject><subject>Biological and medical sciences</subject><subject>Endoplasmic Reticulum - metabolism</subject><subject>Errors of metabolism</subject><subject>Esterification</subject><subject>Fatal Outcome</subject><subject>Fatty Acids, Unsaturated - analysis</subject><subject>Fatty Acids, Unsaturated - metabolism</subject><subject>Female</subject><subject>Humans</subject><subject>Infant</subject><subject>Infant, Newborn</subject><subject>Lipids (lysosomal enzyme disorders, storage diseases)</subject><subject>Liver - chemistry</subject><subject>Liver - metabolism</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Metabolic diseases</subject><subject>Mitochondrial Diseases - metabolism</subject><subject>Muscle, Skeletal - chemistry</subject><subject>Muscle, Skeletal - metabolism</subject><subject>Myocardium - chemistry</subject><subject>Myocardium - metabolism</subject><subject>Oxidation-Reduction</subject><subject>Phospholipids - metabolism</subject><subject>Triglycerides - metabolism</subject><issn>0141-8955</issn><issn>1573-2665</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2001</creationdate><recordtype>article</recordtype><recordid>eNqF0U2OFCEUB3BiNE47unZn2OiuHD6rwF1nHLXNGDe6rlB82JgqKIHKTO_mCN7ExIN4CE8ibXfSceWCEMKP_yPvAfAUo5cYEXqxfoURRlwSQgnF3T2wwryjDWlbfh-sEGa4EZLzM_Ao568IISk4fwjOMGaiFVKswI9NKDZN1nhVbIbRwSVkVZZUjwY6VcoOKu0NjLfeqOJjgCpZ6IOOaY4H5QMsyX8Zd9omb2rKsBQYYtlfzNuY6xr97E3-K33OSzUuxQnONdGGkuGNL1s4-RL1NgaTvBrhr5-_776fqhrrrC75MXjg1Jjtk-N-Dj6_ufp0-a65_vh2c7m-bjRtZddo0VHdcSEl14hJ1jqiiKZ20FgYRRQaHMJtZ4VkRpKOMqa0sVpQhwxvB0vPwYtD7pzit_rf0k8-azuOKti45L7bd53jtsKLA9Qp5pys6-fkJ5V2PUb9fkb9uv9nRvXFs2P0MtTGn_xxKBU8PwKVtRpdUkH7fHIMSc44q44f3I0f7e5_dfv3mw-vEaUd_QM6uK-1</recordid><startdate>200106</startdate><enddate>200106</enddate><creator>Onkenhout, W.</creator><creator>Venizelos, V.</creator><creator>Scholte, H. R.</creator><creator>Klerk, J. B. C.</creator><creator>Poorthuis, B. J. H. M.</creator><general>Kluwer Academic Publishers</general><general>Springer</general><scope>IQODW</scope><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>7X8</scope></search><sort><creationdate>200106</creationdate><title>Intermediates of unsaturated fatty acid oxidation are incorporated in triglycerides but not in phospholipids in tissues from patients with mitochondrial β‐oxidation defects</title><author>Onkenhout, W. ; Venizelos, V. ; Scholte, H. R. ; Klerk, J. B. C. ; Poorthuis, B. J. H. M.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3697-c873c758995c04946f2a2c3ebc18da2a0bf0167e894d927344acdec83f0d56be3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2001</creationdate><topic>Acyl-CoA Dehydrogenase, Long-Chain - deficiency</topic><topic>Biological and medical sciences</topic><topic>Endoplasmic Reticulum - metabolism</topic><topic>Errors of metabolism</topic><topic>Esterification</topic><topic>Fatal Outcome</topic><topic>Fatty Acids, Unsaturated - analysis</topic><topic>Fatty Acids, Unsaturated - metabolism</topic><topic>Female</topic><topic>Humans</topic><topic>Infant</topic><topic>Infant, Newborn</topic><topic>Lipids (lysosomal enzyme disorders, storage diseases)</topic><topic>Liver - chemistry</topic><topic>Liver - metabolism</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Metabolic diseases</topic><topic>Mitochondrial Diseases - metabolism</topic><topic>Muscle, Skeletal - chemistry</topic><topic>Muscle, Skeletal - metabolism</topic><topic>Myocardium - chemistry</topic><topic>Myocardium - metabolism</topic><topic>Oxidation-Reduction</topic><topic>Phospholipids - metabolism</topic><topic>Triglycerides - metabolism</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Onkenhout, W.</creatorcontrib><creatorcontrib>Venizelos, V.</creatorcontrib><creatorcontrib>Scholte, H. R.</creatorcontrib><creatorcontrib>Klerk, J. B. C.</creatorcontrib><creatorcontrib>Poorthuis, B. J. H. M.</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Journal of inherited metabolic disease</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Onkenhout, W.</au><au>Venizelos, V.</au><au>Scholte, H. R.</au><au>Klerk, J. B. C.</au><au>Poorthuis, B. J. H. M.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Intermediates of unsaturated fatty acid oxidation are incorporated in triglycerides but not in phospholipids in tissues from patients with mitochondrial β‐oxidation defects</atitle><jtitle>Journal of inherited metabolic disease</jtitle><addtitle>J Inherit Metab Dis</addtitle><date>2001-06</date><risdate>2001</risdate><volume>24</volume><issue>3</issue><spage>337</spage><epage>344</epage><pages>337-344</pages><issn>0141-8955</issn><eissn>1573-2665</eissn><coden>JIMDDP</coden><abstract>The fatty acid composition was determined of liver, skeletal muscle and heart obtained post mortem from patients with medium‐chain acyl‐CoA dehydrogenase deficiency (MCADD), multiple acyl‐CoA dehydrogenase deficiency (MADD) and very long‐chain acyl‐CoA dehydrogenase deficiency (VLCADD). Increased amounts of 4‐decenoic acid 10:1(n−6), 5‐dodecenoic acid 12:1(n−7), 5‐tetradecenoic acid 14:1(n−9), 5,8‐tetradecadienoic acid 14:2(n−6) and 7,10‐hexadecadienoic acid 16:2(n−6)—intermediates of unsaturated fatty acid oxidation—were found. Fractionation into different lipid classes showed that these fatty acids were exclusively present in the triglyceride fraction. They could not be detected in the free fatty acid fraction or in the phospholipid fraction. Our results suggest that intermediates of unsaturated fatty acid oxidation that accumulate as a consequence of MCADD, MADD and VLCADD are transported to the endoplasmic reticulum for esterification into neutral glycerolipids. The pattern of accumulation is characteristic for each disease, which makes fatty acid analysis of total lipid of post‐mortem tissues a useful tool in the detection of mitochondrial fatty acid oxidation defects in patients who died unexpected, for example with sudden infant death syndrome.</abstract><cop>Dordrecht</cop><pub>Kluwer Academic Publishers</pub><pmid>11486898</pmid><doi>10.1023/A:1010592232317</doi><tpages>8</tpages></addata></record> |
fulltext | fulltext |
identifier | ISSN: 0141-8955 |
ispartof | Journal of inherited metabolic disease, 2001-06, Vol.24 (3), p.337-344 |
issn | 0141-8955 1573-2665 |
language | eng |
recordid | cdi_proquest_miscellaneous_71059516 |
source | Springer Journals; Wiley-Blackwell Read & Publish Collection |
subjects | Acyl-CoA Dehydrogenase, Long-Chain - deficiency Biological and medical sciences Endoplasmic Reticulum - metabolism Errors of metabolism Esterification Fatal Outcome Fatty Acids, Unsaturated - analysis Fatty Acids, Unsaturated - metabolism Female Humans Infant Infant, Newborn Lipids (lysosomal enzyme disorders, storage diseases) Liver - chemistry Liver - metabolism Male Medical sciences Metabolic diseases Mitochondrial Diseases - metabolism Muscle, Skeletal - chemistry Muscle, Skeletal - metabolism Myocardium - chemistry Myocardium - metabolism Oxidation-Reduction Phospholipids - metabolism Triglycerides - metabolism |
title | Intermediates of unsaturated fatty acid oxidation are incorporated in triglycerides but not in phospholipids in tissues from patients with mitochondrial β‐oxidation defects |
url | http://sfxeu10.hosted.exlibrisgroup.com/loughborough?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-03T01%3A22%3A58IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_cross&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Intermediates%20of%20unsaturated%20fatty%20acid%20oxidation%20are%20incorporated%20in%20triglycerides%20but%20not%20in%20phospholipids%20in%20tissues%20from%20patients%20with%20mitochondrial%20%CE%B2%E2%80%90oxidation%20defects&rft.jtitle=Journal%20of%20inherited%20metabolic%20disease&rft.au=Onkenhout,%20W.&rft.date=2001-06&rft.volume=24&rft.issue=3&rft.spage=337&rft.epage=344&rft.pages=337-344&rft.issn=0141-8955&rft.eissn=1573-2665&rft.coden=JIMDDP&rft_id=info:doi/10.1023/A:1010592232317&rft_dat=%3Cproquest_cross%3E71059516%3C/proquest_cross%3E%3Cgrp_id%3Ecdi_FETCH-LOGICAL-c3697-c873c758995c04946f2a2c3ebc18da2a0bf0167e894d927344acdec83f0d56be3%3C/grp_id%3E%3Coa%3E%3C/oa%3E%3Curl%3E%3C/url%3E&rft_id=info:oai/&rft_pqid=71059516&rft_id=info:pmid/11486898&rfr_iscdi=true |