The Mitochondrial Carnitine Palmitoyltransferase System — From Concept to Molecular Analysis

First conceptualized as a mechanism for the mitochondrial transport of long‐chain fatty acids in the early 1960s, the carnitine palmitoyltransferase (CPT) system has since come to be recognized as a pivotal component of fuel homeostasis. This is by virtue of the unique sensitivity of the outer membr...

Ausführliche Beschreibung

Gespeichert in:
Bibliographische Detailangaben
Veröffentlicht in:European journal of biochemistry 1997-02, Vol.244 (1), p.1-14
Hauptverfasser: McGarry, J. Denis, Brown, Nicholas F.
Format: Artikel
Sprache:eng
Schlagworte:
Online-Zugang:Volltext
Tags: Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
container_end_page 14
container_issue 1
container_start_page 1
container_title European journal of biochemistry
container_volume 244
creator McGarry, J. Denis
Brown, Nicholas F.
description First conceptualized as a mechanism for the mitochondrial transport of long‐chain fatty acids in the early 1960s, the carnitine palmitoyltransferase (CPT) system has since come to be recognized as a pivotal component of fuel homeostasis. This is by virtue of the unique sensitivity of the outer membrane CPT I to the simple molecule, malonyl‐CoA. In addition, both CPT I and the inner membrane enzyme, CPT II, have proved to be loci of inherited defects, some with disastrous consequences. Early efforts using classical approaches to characterize the CPT proteins in terms of structure/function/regulatory relationships gave rise to confusion and protracted debate. By contrast, recent application of molecular biological tools has brought major enlightenment at an exponential pace. Here we review some key developments of the last 20 years that have led to our current understanding of the physiology of the CPT system, the structure of the CPT isoforms, the chromosomal localization of their respective genes, and the identification of mutations in the human population.
doi_str_mv 10.1111/j.1432-1033.1997.00001.x
format Article
fullrecord <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_78872699</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>78872699</sourcerecordid><originalsourceid>FETCH-LOGICAL-c4281-2d36b78d1ed888c5c59ae789452ec9121b57a7d686323279757029e95988cce53</originalsourceid><addsrcrecordid>eNqNkM1KxDAQx4Moun48gpCTt9YmaZsET7rsqqAoqFdDNp3FLmmzJl3c3nwIn9AnMXUXz85lYP4fAz-EMMlSEud8kZKc0YRkjKVESp5mcUi63kGjP2EXjeItT6gsygN0GMIiekpZ8n20L7OS5UyO0OvzG-D7unPmzbWVr7XFY-3buqtbwI_aNlHqbed1G-bgdQD81IcOGvz9-YWn3jV47FoDyw53Dt87C2ZltceXrbZ9qMMx2ptrG-Bku4_Qy3TyPL5J7h6ub8eXd4nJqSAJrVg546IiUAkhTGEKqYELmRcUjCSUzAqueVWKklFGueQFz6gEWcjoNlCwI3S26V16976C0KmmDgas1S24VVBcCE5LKaNRbIzGuxA8zNXS1432vSKZGtCqhRoIqoGgGtCqX7RqHaOn2x-rWQPVX3DLMuoXG_2jttD_u1dNJ1dPhP0A5YCH0Q</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>78872699</pqid></control><display><type>article</type><title>The Mitochondrial Carnitine Palmitoyltransferase System — From Concept to Molecular Analysis</title><source>MEDLINE</source><source>Wiley Online Library Journals Frontfile Complete</source><source>Alma/SFX Local Collection</source><creator>McGarry, J. Denis ; Brown, Nicholas F.</creator><creatorcontrib>McGarry, J. Denis ; Brown, Nicholas F.</creatorcontrib><description>First conceptualized as a mechanism for the mitochondrial transport of long‐chain fatty acids in the early 1960s, the carnitine palmitoyltransferase (CPT) system has since come to be recognized as a pivotal component of fuel homeostasis. This is by virtue of the unique sensitivity of the outer membrane CPT I to the simple molecule, malonyl‐CoA. In addition, both CPT I and the inner membrane enzyme, CPT II, have proved to be loci of inherited defects, some with disastrous consequences. Early efforts using classical approaches to characterize the CPT proteins in terms of structure/function/regulatory relationships gave rise to confusion and protracted debate. By contrast, recent application of molecular biological tools has brought major enlightenment at an exponential pace. Here we review some key developments of the last 20 years that have led to our current understanding of the physiology of the CPT system, the structure of the CPT isoforms, the chromosomal localization of their respective genes, and the identification of mutations in the human population.</description><identifier>ISSN: 0014-2956</identifier><identifier>EISSN: 1432-1033</identifier><identifier>DOI: 10.1111/j.1432-1033.1997.00001.x</identifier><identifier>PMID: 9063439</identifier><language>eng</language><publisher>Oxford, UK: Blackwell Science Ltd</publisher><subject>Amino Acid Sequence ; Animals ; Carnitine O-Palmitoyltransferase - metabolism ; Carnitine O-Palmitoyltransferase - physiology ; carnitine palmitoyltransferase ; chromosome mapping ; fuel homeostasis ; Humans ; insulin secretion ; isoform ; malonyl‐CoA ; Mitochondria - enzymology ; mitochondrial protein ; Molecular Sequence Data ; mutation ; pancreatic β‐cell ; tissue distribution ; β‐oxidation</subject><ispartof>European journal of biochemistry, 1997-02, Vol.244 (1), p.1-14</ispartof><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4281-2d36b78d1ed888c5c59ae789452ec9121b57a7d686323279757029e95988cce53</citedby><cites>FETCH-LOGICAL-c4281-2d36b78d1ed888c5c59ae789452ec9121b57a7d686323279757029e95988cce53</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1111%2Fj.1432-1033.1997.00001.x$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1111%2Fj.1432-1033.1997.00001.x$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,776,780,1411,27901,27902,45550,45551</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/9063439$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>McGarry, J. Denis</creatorcontrib><creatorcontrib>Brown, Nicholas F.</creatorcontrib><title>The Mitochondrial Carnitine Palmitoyltransferase System — From Concept to Molecular Analysis</title><title>European journal of biochemistry</title><addtitle>Eur J Biochem</addtitle><description>First conceptualized as a mechanism for the mitochondrial transport of long‐chain fatty acids in the early 1960s, the carnitine palmitoyltransferase (CPT) system has since come to be recognized as a pivotal component of fuel homeostasis. This is by virtue of the unique sensitivity of the outer membrane CPT I to the simple molecule, malonyl‐CoA. In addition, both CPT I and the inner membrane enzyme, CPT II, have proved to be loci of inherited defects, some with disastrous consequences. Early efforts using classical approaches to characterize the CPT proteins in terms of structure/function/regulatory relationships gave rise to confusion and protracted debate. By contrast, recent application of molecular biological tools has brought major enlightenment at an exponential pace. Here we review some key developments of the last 20 years that have led to our current understanding of the physiology of the CPT system, the structure of the CPT isoforms, the chromosomal localization of their respective genes, and the identification of mutations in the human population.</description><subject>Amino Acid Sequence</subject><subject>Animals</subject><subject>Carnitine O-Palmitoyltransferase - metabolism</subject><subject>Carnitine O-Palmitoyltransferase - physiology</subject><subject>carnitine palmitoyltransferase</subject><subject>chromosome mapping</subject><subject>fuel homeostasis</subject><subject>Humans</subject><subject>insulin secretion</subject><subject>isoform</subject><subject>malonyl‐CoA</subject><subject>Mitochondria - enzymology</subject><subject>mitochondrial protein</subject><subject>Molecular Sequence Data</subject><subject>mutation</subject><subject>pancreatic β‐cell</subject><subject>tissue distribution</subject><subject>β‐oxidation</subject><issn>0014-2956</issn><issn>1432-1033</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1997</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNkM1KxDAQx4Moun48gpCTt9YmaZsET7rsqqAoqFdDNp3FLmmzJl3c3nwIn9AnMXUXz85lYP4fAz-EMMlSEud8kZKc0YRkjKVESp5mcUi63kGjP2EXjeItT6gsygN0GMIiekpZ8n20L7OS5UyO0OvzG-D7unPmzbWVr7XFY-3buqtbwI_aNlHqbed1G-bgdQD81IcOGvz9-YWn3jV47FoDyw53Dt87C2ZltceXrbZ9qMMx2ptrG-Bku4_Qy3TyPL5J7h6ub8eXd4nJqSAJrVg546IiUAkhTGEKqYELmRcUjCSUzAqueVWKklFGueQFz6gEWcjoNlCwI3S26V16976C0KmmDgas1S24VVBcCE5LKaNRbIzGuxA8zNXS1432vSKZGtCqhRoIqoGgGtCqX7RqHaOn2x-rWQPVX3DLMuoXG_2jttD_u1dNJ1dPhP0A5YCH0Q</recordid><startdate>19970215</startdate><enddate>19970215</enddate><creator>McGarry, J. Denis</creator><creator>Brown, Nicholas F.</creator><general>Blackwell Science Ltd</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>7X8</scope></search><sort><creationdate>19970215</creationdate><title>The Mitochondrial Carnitine Palmitoyltransferase System — From Concept to Molecular Analysis</title><author>McGarry, J. Denis ; Brown, Nicholas F.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4281-2d36b78d1ed888c5c59ae789452ec9121b57a7d686323279757029e95988cce53</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1997</creationdate><topic>Amino Acid Sequence</topic><topic>Animals</topic><topic>Carnitine O-Palmitoyltransferase - metabolism</topic><topic>Carnitine O-Palmitoyltransferase - physiology</topic><topic>carnitine palmitoyltransferase</topic><topic>chromosome mapping</topic><topic>fuel homeostasis</topic><topic>Humans</topic><topic>insulin secretion</topic><topic>isoform</topic><topic>malonyl‐CoA</topic><topic>Mitochondria - enzymology</topic><topic>mitochondrial protein</topic><topic>Molecular Sequence Data</topic><topic>mutation</topic><topic>pancreatic β‐cell</topic><topic>tissue distribution</topic><topic>β‐oxidation</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>McGarry, J. Denis</creatorcontrib><creatorcontrib>Brown, Nicholas F.</creatorcontrib><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>European journal of biochemistry</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>McGarry, J. Denis</au><au>Brown, Nicholas F.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>The Mitochondrial Carnitine Palmitoyltransferase System — From Concept to Molecular Analysis</atitle><jtitle>European journal of biochemistry</jtitle><addtitle>Eur J Biochem</addtitle><date>1997-02-15</date><risdate>1997</risdate><volume>244</volume><issue>1</issue><spage>1</spage><epage>14</epage><pages>1-14</pages><issn>0014-2956</issn><eissn>1432-1033</eissn><abstract>First conceptualized as a mechanism for the mitochondrial transport of long‐chain fatty acids in the early 1960s, the carnitine palmitoyltransferase (CPT) system has since come to be recognized as a pivotal component of fuel homeostasis. This is by virtue of the unique sensitivity of the outer membrane CPT I to the simple molecule, malonyl‐CoA. In addition, both CPT I and the inner membrane enzyme, CPT II, have proved to be loci of inherited defects, some with disastrous consequences. Early efforts using classical approaches to characterize the CPT proteins in terms of structure/function/regulatory relationships gave rise to confusion and protracted debate. By contrast, recent application of molecular biological tools has brought major enlightenment at an exponential pace. Here we review some key developments of the last 20 years that have led to our current understanding of the physiology of the CPT system, the structure of the CPT isoforms, the chromosomal localization of their respective genes, and the identification of mutations in the human population.</abstract><cop>Oxford, UK</cop><pub>Blackwell Science Ltd</pub><pmid>9063439</pmid><doi>10.1111/j.1432-1033.1997.00001.x</doi><tpages>14</tpages></addata></record>
fulltext fulltext
identifier ISSN: 0014-2956
ispartof European journal of biochemistry, 1997-02, Vol.244 (1), p.1-14
issn 0014-2956
1432-1033
language eng
recordid cdi_proquest_miscellaneous_78872699
source MEDLINE; Wiley Online Library Journals Frontfile Complete; Alma/SFX Local Collection
subjects Amino Acid Sequence
Animals
Carnitine O-Palmitoyltransferase - metabolism
Carnitine O-Palmitoyltransferase - physiology
carnitine palmitoyltransferase
chromosome mapping
fuel homeostasis
Humans
insulin secretion
isoform
malonyl‐CoA
Mitochondria - enzymology
mitochondrial protein
Molecular Sequence Data
mutation
pancreatic β‐cell
tissue distribution
β‐oxidation
title The Mitochondrial Carnitine Palmitoyltransferase System — From Concept to Molecular Analysis
url https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-02-09T03%3A07%3A30IST&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=The%20Mitochondrial%20Carnitine%20Palmitoyltransferase%20System%20%E2%80%94%20From%20Concept%20to%20Molecular%20Analysis&rft.jtitle=European%20journal%20of%20biochemistry&rft.au=McGarry,%20J.%20Denis&rft.date=1997-02-15&rft.volume=244&rft.issue=1&rft.spage=1&rft.epage=14&rft.pages=1-14&rft.issn=0014-2956&rft.eissn=1432-1033&rft_id=info:doi/10.1111/j.1432-1033.1997.00001.x&rft_dat=%3Cproquest_cross%3E78872699%3C/proquest_cross%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=78872699&rft_id=info:pmid/9063439&rfr_iscdi=true