Deficient oxidation of trihydroxycoprostanic acid in liver homogenates from patients with peroxisomal diseases
Summary The activation of palmitate and trihydroxycoprostanic acid and the peroxisomal oxidation of palmitate, trihydroxycoprostanic acid and their CoA esters were measured in homogenates prepared from fresh liver tissue of patients undergoing hepatic surgery and from frozen postmortem liver specime...
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| Veröffentlicht in: | Journal of inherited metabolic disease 1989-12, Vol.12 (4), p.415-422 |
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| container_title | Journal of inherited metabolic disease |
| container_volume | 12 |
| creator | Casteels, M. Van Roermund, C. W. T. Schepers, L. Govaert, L. Eyssen, H. J. Mannaerts, G. P. Wanders, R. J. A. |
| description | Summary
The activation of palmitate and trihydroxycoprostanic acid and the peroxisomal oxidation of palmitate, trihydroxycoprostanic acid and their CoA esters were measured in homogenates prepared from fresh liver tissue of patients undergoing hepatic surgery and from frozen postmortem liver specimens of controls, patients with Zellweger syndrome and a patient with pseudo‐Zellweger syndrome, a deficiency of peroxisomal 3‐oxoacyl‐CoA thiolase. In contrast to the findings in control livers, peroxisomal β‐oxidation of palmitate and of palmitoyl‐CoA was severely impaired, and oxidation of trihydroxycoprostanic acid and its CoA ester could not be detected in the livers of the patients affected by peroxisomal diseases. The finding in this paper, that the oxidation of trihydroxycoprostanoyl‐CoA can be measured reliably in small amounts of human liver, will be of valuable help in the differential diagnosis and classification of peroxisomal disorders and will help to elucidate the exact nature of some of the defects present in these disorders. |
| doi_str_mv | 10.1007/BF01802036 |
| format | Article |
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The activation of palmitate and trihydroxycoprostanic acid and the peroxisomal oxidation of palmitate, trihydroxycoprostanic acid and their CoA esters were measured in homogenates prepared from fresh liver tissue of patients undergoing hepatic surgery and from frozen postmortem liver specimens of controls, patients with Zellweger syndrome and a patient with pseudo‐Zellweger syndrome, a deficiency of peroxisomal 3‐oxoacyl‐CoA thiolase. In contrast to the findings in control livers, peroxisomal β‐oxidation of palmitate and of palmitoyl‐CoA was severely impaired, and oxidation of trihydroxycoprostanic acid and its CoA ester could not be detected in the livers of the patients affected by peroxisomal diseases. The finding in this paper, that the oxidation of trihydroxycoprostanoyl‐CoA can be measured reliably in small amounts of human liver, will be of valuable help in the differential diagnosis and classification of peroxisomal disorders and will help to elucidate the exact nature of some of the defects present in these disorders.</description><identifier>ISSN: 0141-8955</identifier><identifier>EISSN: 1573-2665</identifier><identifier>DOI: 10.1007/BF01802036</identifier><identifier>PMID: 2576087</identifier><identifier>CODEN: JIMDDP</identifier><language>eng</language><publisher>Dordrecht: Kluwer Academic Publishers</publisher><subject>Acetyl-CoA C-Acetyltransferase - deficiency ; Acetyl-CoA C-Acetyltransferase - metabolism ; Acetyltransferases - deficiency ; Biological and medical sciences ; Cholic Acids - metabolism ; Errors of metabolism ; Humans ; Lipid Peroxidation ; Liver - enzymology ; Medical sciences ; Metabolic diseases ; Microbodies - enzymology ; Miscellaneous hereditary metabolic disorders ; Substrate Specificity ; Zellweger Syndrome - enzymology ; Zellweger Syndrome - metabolism</subject><ispartof>Journal of inherited metabolic disease, 1989-12, Vol.12 (4), p.415-422</ispartof><rights>1989 SSIEM</rights><rights>1990 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3525-840e454146e355178b35e47550fa5f1d0427709549cc819e3412349f6867a6de3</citedby><cites>FETCH-LOGICAL-c3525-840e454146e355178b35e47550fa5f1d0427709549cc819e3412349f6867a6de3</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=6791915$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/2576087$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Casteels, M.</creatorcontrib><creatorcontrib>Van Roermund, C. W. T.</creatorcontrib><creatorcontrib>Schepers, L.</creatorcontrib><creatorcontrib>Govaert, L.</creatorcontrib><creatorcontrib>Eyssen, H. J.</creatorcontrib><creatorcontrib>Mannaerts, G. P.</creatorcontrib><creatorcontrib>Wanders, R. J. A.</creatorcontrib><title>Deficient oxidation of trihydroxycoprostanic acid in liver homogenates from patients with peroxisomal diseases</title><title>Journal of inherited metabolic disease</title><addtitle>J Inherit Metab Dis</addtitle><description>Summary
The activation of palmitate and trihydroxycoprostanic acid and the peroxisomal oxidation of palmitate, trihydroxycoprostanic acid and their CoA esters were measured in homogenates prepared from fresh liver tissue of patients undergoing hepatic surgery and from frozen postmortem liver specimens of controls, patients with Zellweger syndrome and a patient with pseudo‐Zellweger syndrome, a deficiency of peroxisomal 3‐oxoacyl‐CoA thiolase. In contrast to the findings in control livers, peroxisomal β‐oxidation of palmitate and of palmitoyl‐CoA was severely impaired, and oxidation of trihydroxycoprostanic acid and its CoA ester could not be detected in the livers of the patients affected by peroxisomal diseases. The finding in this paper, that the oxidation of trihydroxycoprostanoyl‐CoA can be measured reliably in small amounts of human liver, will be of valuable help in the differential diagnosis and classification of peroxisomal disorders and will help to elucidate the exact nature of some of the defects present in these disorders.</description><subject>Acetyl-CoA C-Acetyltransferase - deficiency</subject><subject>Acetyl-CoA C-Acetyltransferase - metabolism</subject><subject>Acetyltransferases - deficiency</subject><subject>Biological and medical sciences</subject><subject>Cholic Acids - metabolism</subject><subject>Errors of metabolism</subject><subject>Humans</subject><subject>Lipid Peroxidation</subject><subject>Liver - enzymology</subject><subject>Medical sciences</subject><subject>Metabolic diseases</subject><subject>Microbodies - enzymology</subject><subject>Miscellaneous hereditary metabolic disorders</subject><subject>Substrate Specificity</subject><subject>Zellweger Syndrome - enzymology</subject><subject>Zellweger Syndrome - metabolism</subject><issn>0141-8955</issn><issn>1573-2665</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1989</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kDFPHDEQhS2UiFyAJn0kF1GKSBvGux57XQYIBESUBuqV8Y5zRrvrw94D7t9jdCfSpZrife_NzGPsk4DvAkAfn5yDaKGGRu2xhUDdVLVS-I4tQEhRtQbxA_uY8z0AmBZxn-3XqBW0esGmM_LBBZpmHp9Db-cQJx49n1NYbvoUnzcurlLMs52C49aFnoeJD-GREl_GMf6lyc6UuU9x5KtiL0mZP4V5yVdU7CHH0Q68D5lspnzI3ns7ZDrazQN2e_7z5vRXdf3n4vL0x3XlGqyxaiWQRCmkogZR6PauQZIaEbxFL3qQtdZgUBrnWmGokaJupPGqVdqqnpoD9nWbW25_WFOeuzFkR8NgJ4rr3GkjtVBCFPDbFnTlyZzId6sURps2nYDutdzuX7kF_rxLXd-N1L-huzaL_mWn2-zs4JOdXMhvmNJGGIEFgy32FAba_Gdhd3X5-wxksbwAmQOPsA</recordid><startdate>198912</startdate><enddate>198912</enddate><creator>Casteels, M.</creator><creator>Van Roermund, C. W. T.</creator><creator>Schepers, L.</creator><creator>Govaert, L.</creator><creator>Eyssen, H. J.</creator><creator>Mannaerts, G. P.</creator><creator>Wanders, R. J. A.</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>198912</creationdate><title>Deficient oxidation of trihydroxycoprostanic acid in liver homogenates from patients with peroxisomal diseases</title><author>Casteels, M. ; Van Roermund, C. W. T. ; Schepers, L. ; Govaert, L. ; Eyssen, H. J. ; Mannaerts, G. P. ; Wanders, R. J. A.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3525-840e454146e355178b35e47550fa5f1d0427709549cc819e3412349f6867a6de3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1989</creationdate><topic>Acetyl-CoA C-Acetyltransferase - deficiency</topic><topic>Acetyl-CoA C-Acetyltransferase - metabolism</topic><topic>Acetyltransferases - deficiency</topic><topic>Biological and medical sciences</topic><topic>Cholic Acids - metabolism</topic><topic>Errors of metabolism</topic><topic>Humans</topic><topic>Lipid Peroxidation</topic><topic>Liver - enzymology</topic><topic>Medical sciences</topic><topic>Metabolic diseases</topic><topic>Microbodies - enzymology</topic><topic>Miscellaneous hereditary metabolic disorders</topic><topic>Substrate Specificity</topic><topic>Zellweger Syndrome - enzymology</topic><topic>Zellweger Syndrome - metabolism</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Casteels, M.</creatorcontrib><creatorcontrib>Van Roermund, C. W. T.</creatorcontrib><creatorcontrib>Schepers, L.</creatorcontrib><creatorcontrib>Govaert, L.</creatorcontrib><creatorcontrib>Eyssen, H. J.</creatorcontrib><creatorcontrib>Mannaerts, G. P.</creatorcontrib><creatorcontrib>Wanders, R. J. A.</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>Casteels, M.</au><au>Van Roermund, C. W. T.</au><au>Schepers, L.</au><au>Govaert, L.</au><au>Eyssen, H. J.</au><au>Mannaerts, G. P.</au><au>Wanders, R. J. A.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Deficient oxidation of trihydroxycoprostanic acid in liver homogenates from patients with peroxisomal diseases</atitle><jtitle>Journal of inherited metabolic disease</jtitle><addtitle>J Inherit Metab Dis</addtitle><date>1989-12</date><risdate>1989</risdate><volume>12</volume><issue>4</issue><spage>415</spage><epage>422</epage><pages>415-422</pages><issn>0141-8955</issn><eissn>1573-2665</eissn><coden>JIMDDP</coden><abstract>Summary
The activation of palmitate and trihydroxycoprostanic acid and the peroxisomal oxidation of palmitate, trihydroxycoprostanic acid and their CoA esters were measured in homogenates prepared from fresh liver tissue of patients undergoing hepatic surgery and from frozen postmortem liver specimens of controls, patients with Zellweger syndrome and a patient with pseudo‐Zellweger syndrome, a deficiency of peroxisomal 3‐oxoacyl‐CoA thiolase. In contrast to the findings in control livers, peroxisomal β‐oxidation of palmitate and of palmitoyl‐CoA was severely impaired, and oxidation of trihydroxycoprostanic acid and its CoA ester could not be detected in the livers of the patients affected by peroxisomal diseases. The finding in this paper, that the oxidation of trihydroxycoprostanoyl‐CoA can be measured reliably in small amounts of human liver, will be of valuable help in the differential diagnosis and classification of peroxisomal disorders and will help to elucidate the exact nature of some of the defects present in these disorders.</abstract><cop>Dordrecht</cop><pub>Kluwer Academic Publishers</pub><pmid>2576087</pmid><doi>10.1007/BF01802036</doi><tpages>8</tpages></addata></record> |
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| subjects | Acetyl-CoA C-Acetyltransferase - deficiency Acetyl-CoA C-Acetyltransferase - metabolism Acetyltransferases - deficiency Biological and medical sciences Cholic Acids - metabolism Errors of metabolism Humans Lipid Peroxidation Liver - enzymology Medical sciences Metabolic diseases Microbodies - enzymology Miscellaneous hereditary metabolic disorders Substrate Specificity Zellweger Syndrome - enzymology Zellweger Syndrome - metabolism |
| title | Deficient oxidation of trihydroxycoprostanic acid in liver homogenates from patients with peroxisomal diseases |
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