Amino-acid depletion induced by abnormal amino-acid conjugation and protein restriction in isovaleric acidemia
Background: Previously, we detected 19 'new' amino-acid conjugates in the urine of patients with isovaleric acidemia. There is currently a poor understanding of the relationship between the clinical symptoms and the excreted metabolites occurring in these patients, owing to insufficient me...
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| Veröffentlicht in: | European journal of clinical nutrition 2007-11, Vol.61 (11), p.1323-1327 |
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| description | Background: Previously, we detected 19 'new' amino-acid conjugates in the urine of patients with isovaleric acidemia. There is currently a poor understanding of the relationship between the clinical symptoms and the excreted metabolites occurring in these patients, owing to insufficient metabolite characterization and quantification. Consequently, controversial treatment protocols exist, particularly pertaining to dietary protein restriction. Objective: To determine the effect of the previously identified amino-acid conjugates and conventional dietary protein restriction therapy, on the free amino-acid concentrations in isovaleric acidemia patients, to better explain the clinical symptoms and develop more effective therapy. Design: Free amino-acid quantification via liquid chromatography mass spectrometry (LC-MS-MS) was performed on pre- and post-treatment urine or serum samples collected from six isovaleric acidemia patients, previously investigated for the presence of new induced N-isovaleryl and N-acetyl-amino-acid conjugates. Results: Depleted amino-acid concentrations were detected in varying degrees in all six patients and did not recover after conventional treatment. Conclusions: The 19 potentially toxic metabolites previously identified and the consequent amino-acid depletions detected in this study, may explain many of the clinical symptoms associated with isovaleric acidemia. Furthermore, the occurrence of amino-acid depletions in these patients, steers away from the controversial dietary protein restriction treatment protocols, and towards dietary leucine restriction alone with essential amino-acid supplementation, in combination with glycine and L-carnitine supplementation. |
| doi_str_mv | 10.1038/sj.ejcn.1602648 |
| format | Article |
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There is currently a poor understanding of the relationship between the clinical symptoms and the excreted metabolites occurring in these patients, owing to insufficient metabolite characterization and quantification. Consequently, controversial treatment protocols exist, particularly pertaining to dietary protein restriction. Objective: To determine the effect of the previously identified amino-acid conjugates and conventional dietary protein restriction therapy, on the free amino-acid concentrations in isovaleric acidemia patients, to better explain the clinical symptoms and develop more effective therapy. Design: Free amino-acid quantification via liquid chromatography mass spectrometry (LC-MS-MS) was performed on pre- and post-treatment urine or serum samples collected from six isovaleric acidemia patients, previously investigated for the presence of new induced N-isovaleryl and N-acetyl-amino-acid conjugates. Results: Depleted amino-acid concentrations were detected in varying degrees in all six patients and did not recover after conventional treatment. Conclusions: The 19 potentially toxic metabolites previously identified and the consequent amino-acid depletions detected in this study, may explain many of the clinical symptoms associated with isovaleric acidemia. Furthermore, the occurrence of amino-acid depletions in these patients, steers away from the controversial dietary protein restriction treatment protocols, and towards dietary leucine restriction alone with essential amino-acid supplementation, in combination with glycine and L-carnitine supplementation.</description><identifier>ISSN: 0954-3007</identifier><identifier>EISSN: 1476-5640</identifier><identifier>DOI: 10.1038/sj.ejcn.1602648</identifier><identifier>PMID: 17299485</identifier><language>eng</language><publisher>London: Nature Publishing Group UK</publisher><subject>Acids ; Amino Acid Metabolism, Inborn Errors - blood ; Amino Acid Metabolism, Inborn Errors - diet therapy ; Amino Acid Metabolism, Inborn Errors - urine ; Amino acids ; Amino Acids - blood ; Amino Acids - metabolism ; Amino Acids - urine ; Aminoacid disorders ; Biological and medical sciences ; Carnitine ; Clinical Nutrition ; Conjugates ; Conjugation ; Depletion ; Diet ; diet therapy ; Diet, Protein-Restricted ; diet-related diseases ; dietary protein ; dietary protein restriction therapy ; Dietary Proteins - administration & dosage ; dietary restriction ; Dietary restrictions ; Dietary supplements ; Epidemiology ; Errors of metabolism ; excretion ; free amino acids ; Glycine ; glycine (amino acid) ; Humans ; Infant, Newborn ; Internal Medicine ; isovaleric acid ; isovaleric acidemia ; Leucine ; leucine restriction ; Liquid chromatography ; Mass spectrometry ; Mass spectroscopy ; Medical sciences ; Medicine ; Medicine & Public Health ; Metabolic Diseases ; Metabolites ; nutrient intake ; nutritional intervention ; original-article ; Patients ; Pentanoic Acids - blood ; Pentanoic Acids - metabolism ; Pentanoic Acids - urine ; protein supplements ; Proteins ; Public Health ; signs and symptoms (animals and humans) ; special diets ; Spectrometry, Mass, Electrospray Ionization - methods ; Treatment Outcome ; Urine ; valeric acid</subject><ispartof>European journal of clinical nutrition, 2007-11, Vol.61 (11), p.1323-1327</ispartof><rights>Springer Nature Limited 2007</rights><rights>2008 INIST-CNRS</rights><rights>COPYRIGHT 2007 Nature Publishing Group</rights><rights>Copyright Nature Publishing Group Nov 2007</rights><rights>Nature Publishing Group 2007.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c575t-bb49523d155d0196f5c59259a87454c0bd234048f1d13ce4862e6bdc24d10a793</citedby><cites>FETCH-LOGICAL-c575t-bb49523d155d0196f5c59259a87454c0bd234048f1d13ce4862e6bdc24d10a793</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,2727,27924,27925</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=19198476$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/17299485$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Loots, D.T</creatorcontrib><creatorcontrib>Mienie, L.J</creatorcontrib><creatorcontrib>Erasmus, E</creatorcontrib><title>Amino-acid depletion induced by abnormal amino-acid conjugation and protein restriction in isovaleric acidemia</title><title>European journal of clinical nutrition</title><addtitle>Eur J Clin Nutr</addtitle><addtitle>Eur J Clin Nutr</addtitle><description>Background: Previously, we detected 19 'new' amino-acid conjugates in the urine of patients with isovaleric acidemia. There is currently a poor understanding of the relationship between the clinical symptoms and the excreted metabolites occurring in these patients, owing to insufficient metabolite characterization and quantification. Consequently, controversial treatment protocols exist, particularly pertaining to dietary protein restriction. Objective: To determine the effect of the previously identified amino-acid conjugates and conventional dietary protein restriction therapy, on the free amino-acid concentrations in isovaleric acidemia patients, to better explain the clinical symptoms and develop more effective therapy. Design: Free amino-acid quantification via liquid chromatography mass spectrometry (LC-MS-MS) was performed on pre- and post-treatment urine or serum samples collected from six isovaleric acidemia patients, previously investigated for the presence of new induced N-isovaleryl and N-acetyl-amino-acid conjugates. Results: Depleted amino-acid concentrations were detected in varying degrees in all six patients and did not recover after conventional treatment. Conclusions: The 19 potentially toxic metabolites previously identified and the consequent amino-acid depletions detected in this study, may explain many of the clinical symptoms associated with isovaleric acidemia. Furthermore, the occurrence of amino-acid depletions in these patients, steers away from the controversial dietary protein restriction treatment protocols, and towards dietary leucine restriction alone with essential amino-acid supplementation, in combination with glycine and L-carnitine supplementation.</description><subject>Acids</subject><subject>Amino Acid Metabolism, Inborn Errors - blood</subject><subject>Amino Acid Metabolism, Inborn Errors - diet therapy</subject><subject>Amino Acid Metabolism, Inborn Errors - urine</subject><subject>Amino acids</subject><subject>Amino Acids - blood</subject><subject>Amino Acids - metabolism</subject><subject>Amino Acids - urine</subject><subject>Aminoacid disorders</subject><subject>Biological and medical sciences</subject><subject>Carnitine</subject><subject>Clinical Nutrition</subject><subject>Conjugates</subject><subject>Conjugation</subject><subject>Depletion</subject><subject>Diet</subject><subject>diet therapy</subject><subject>Diet, Protein-Restricted</subject><subject>diet-related diseases</subject><subject>dietary protein</subject><subject>dietary protein restriction therapy</subject><subject>Dietary Proteins - administration & dosage</subject><subject>dietary restriction</subject><subject>Dietary restrictions</subject><subject>Dietary supplements</subject><subject>Epidemiology</subject><subject>Errors of metabolism</subject><subject>excretion</subject><subject>free amino acids</subject><subject>Glycine</subject><subject>glycine (amino acid)</subject><subject>Humans</subject><subject>Infant, Newborn</subject><subject>Internal Medicine</subject><subject>isovaleric acid</subject><subject>isovaleric acidemia</subject><subject>Leucine</subject><subject>leucine restriction</subject><subject>Liquid chromatography</subject><subject>Mass spectrometry</subject><subject>Mass spectroscopy</subject><subject>Medical sciences</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Metabolic Diseases</subject><subject>Metabolites</subject><subject>nutrient intake</subject><subject>nutritional intervention</subject><subject>original-article</subject><subject>Patients</subject><subject>Pentanoic Acids - blood</subject><subject>Pentanoic Acids - metabolism</subject><subject>Pentanoic Acids - urine</subject><subject>protein supplements</subject><subject>Proteins</subject><subject>Public Health</subject><subject>signs and symptoms (animals and humans)</subject><subject>special diets</subject><subject>Spectrometry, Mass, Electrospray Ionization - methods</subject><subject>Treatment Outcome</subject><subject>Urine</subject><subject>valeric acid</subject><issn>0954-3007</issn><issn>1476-5640</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2007</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>8G5</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><sourceid>GUQSH</sourceid><sourceid>M2O</sourceid><recordid>eNp1kk1v1DAQhiMEokvhzAmIQHDL1nbsJD6uqhaQKnGAnqOJ7aSOsvZiJ0j990xIREBq5YMlz_POl98keU3JnpK8uoj93vTK7WlBWMGrJ8mO8rLIRMHJ02RHpOBZTkh5lryIsScEgyV7npzRkknJK7FL3OFonc9AWZ1qcxrMaL1LrdOTMjpt7lNonA9HGFLYQOVdP3XwBwWn01Pwo7EuDSaOwao1RWqj_wWDwZd0lpmjhZfJsxaGaF6t93lye3314_JLdvPt89fLw02mRCnGrGm4FCzXVAhNqCxaoYRkQkJVcsEVaTTLOeFVSzXNleFVwUzRaMW4pgRKmZ8nH5a82NrPCduqez8FhyVr3BMrWMkrgtT7RymsWpScMISyBepwltq61o8BVGecCTB4Z1qLzwcqucSOaIX8_gEez7wA9aDg0z-COwPDeBf9MM1rjP-DFwuogo8xmLY-BXuEcF9TUs92qGNfz3aoVzug4u064NQcjd749f8R-LgCEBUMbQCnbNw4SWWFhkKOLFzEkOtM2Db1eO03i8TBOAWz5fwbf7fEW_A1dAHL3n5nhKJfK54XhOS_AQSG35s</recordid><startdate>20071101</startdate><enddate>20071101</enddate><creator>Loots, D.T</creator><creator>Mienie, L.J</creator><creator>Erasmus, E</creator><general>Nature Publishing Group UK</general><general>Nature Publishing</general><general>Nature Publishing Group</general><scope>FBQ</scope><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>3V.</scope><scope>7QP</scope><scope>7RV</scope><scope>7TK</scope><scope>7X2</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8C1</scope><scope>8FE</scope><scope>8FH</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>8G5</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AN0</scope><scope>ATCPS</scope><scope>AZQEC</scope><scope>BBNVY</scope><scope>BENPR</scope><scope>BHPHI</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>GUQSH</scope><scope>HCIFZ</scope><scope>K9.</scope><scope>KB0</scope><scope>LK8</scope><scope>M0K</scope><scope>M0S</scope><scope>M1P</scope><scope>M2O</scope><scope>M7P</scope><scope>MBDVC</scope><scope>NAPCQ</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>Q9U</scope></search><sort><creationdate>20071101</creationdate><title>Amino-acid depletion induced by abnormal amino-acid conjugation and protein restriction in isovaleric acidemia</title><author>Loots, D.T ; Mienie, L.J ; Erasmus, E</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c575t-bb49523d155d0196f5c59259a87454c0bd234048f1d13ce4862e6bdc24d10a793</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2007</creationdate><topic>Acids</topic><topic>Amino Acid Metabolism, Inborn Errors - blood</topic><topic>Amino Acid Metabolism, Inborn Errors - diet therapy</topic><topic>Amino Acid Metabolism, Inborn Errors - urine</topic><topic>Amino acids</topic><topic>Amino Acids - blood</topic><topic>Amino Acids - metabolism</topic><topic>Amino Acids - urine</topic><topic>Aminoacid disorders</topic><topic>Biological and medical sciences</topic><topic>Carnitine</topic><topic>Clinical Nutrition</topic><topic>Conjugates</topic><topic>Conjugation</topic><topic>Depletion</topic><topic>Diet</topic><topic>diet therapy</topic><topic>Diet, Protein-Restricted</topic><topic>diet-related diseases</topic><topic>dietary protein</topic><topic>dietary protein restriction therapy</topic><topic>Dietary Proteins - administration & dosage</topic><topic>dietary restriction</topic><topic>Dietary restrictions</topic><topic>Dietary supplements</topic><topic>Epidemiology</topic><topic>Errors of metabolism</topic><topic>excretion</topic><topic>free amino acids</topic><topic>Glycine</topic><topic>glycine (amino acid)</topic><topic>Humans</topic><topic>Infant, Newborn</topic><topic>Internal Medicine</topic><topic>isovaleric acid</topic><topic>isovaleric acidemia</topic><topic>Leucine</topic><topic>leucine restriction</topic><topic>Liquid chromatography</topic><topic>Mass spectrometry</topic><topic>Mass spectroscopy</topic><topic>Medical sciences</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Metabolic Diseases</topic><topic>Metabolites</topic><topic>nutrient intake</topic><topic>nutritional intervention</topic><topic>original-article</topic><topic>Patients</topic><topic>Pentanoic Acids - blood</topic><topic>Pentanoic Acids - metabolism</topic><topic>Pentanoic Acids - urine</topic><topic>protein supplements</topic><topic>Proteins</topic><topic>Public Health</topic><topic>signs and symptoms (animals and humans)</topic><topic>special diets</topic><topic>Spectrometry, Mass, Electrospray Ionization - methods</topic><topic>Treatment Outcome</topic><topic>Urine</topic><topic>valeric acid</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Loots, D.T</creatorcontrib><creatorcontrib>Mienie, L.J</creatorcontrib><creatorcontrib>Erasmus, E</creatorcontrib><collection>AGRIS</collection><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>ProQuest Central (Corporate)</collection><collection>Calcium & Calcified Tissue Abstracts</collection><collection>Nursing & Allied Health Database</collection><collection>Neurosciences Abstracts</collection><collection>Agricultural Science Collection</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>ProQuest Pharma Collection</collection><collection>Public Health 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>Research Library (Alumni Edition)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>British Nursing Database</collection><collection>Agricultural & Environmental Science Collection</collection><collection>ProQuest Central Essentials</collection><collection>Biological Science Collection</collection><collection>ProQuest Central</collection><collection>Natural Science Collection</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central Korea</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Central Student</collection><collection>Research Library Prep</collection><collection>SciTech Premium Collection</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Database (Alumni Edition)</collection><collection>ProQuest Biological Science Collection</collection><collection>Agricultural Science Database</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Research Library</collection><collection>Biological Science Database</collection><collection>Research Library (Corporate)</collection><collection>Nursing & Allied Health Premium</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><jtitle>European journal of clinical nutrition</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Loots, D.T</au><au>Mienie, L.J</au><au>Erasmus, E</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Amino-acid depletion induced by abnormal amino-acid conjugation and protein restriction in isovaleric acidemia</atitle><jtitle>European journal of clinical nutrition</jtitle><stitle>Eur J Clin Nutr</stitle><addtitle>Eur J Clin Nutr</addtitle><date>2007-11-01</date><risdate>2007</risdate><volume>61</volume><issue>11</issue><spage>1323</spage><epage>1327</epage><pages>1323-1327</pages><issn>0954-3007</issn><eissn>1476-5640</eissn><abstract>Background: Previously, we detected 19 'new' amino-acid conjugates in the urine of patients with isovaleric acidemia. There is currently a poor understanding of the relationship between the clinical symptoms and the excreted metabolites occurring in these patients, owing to insufficient metabolite characterization and quantification. Consequently, controversial treatment protocols exist, particularly pertaining to dietary protein restriction. Objective: To determine the effect of the previously identified amino-acid conjugates and conventional dietary protein restriction therapy, on the free amino-acid concentrations in isovaleric acidemia patients, to better explain the clinical symptoms and develop more effective therapy. Design: Free amino-acid quantification via liquid chromatography mass spectrometry (LC-MS-MS) was performed on pre- and post-treatment urine or serum samples collected from six isovaleric acidemia patients, previously investigated for the presence of new induced N-isovaleryl and N-acetyl-amino-acid conjugates. Results: Depleted amino-acid concentrations were detected in varying degrees in all six patients and did not recover after conventional treatment. Conclusions: The 19 potentially toxic metabolites previously identified and the consequent amino-acid depletions detected in this study, may explain many of the clinical symptoms associated with isovaleric acidemia. Furthermore, the occurrence of amino-acid depletions in these patients, steers away from the controversial dietary protein restriction treatment protocols, and towards dietary leucine restriction alone with essential amino-acid supplementation, in combination with glycine and L-carnitine supplementation.</abstract><cop>London</cop><pub>Nature Publishing Group UK</pub><pmid>17299485</pmid><doi>10.1038/sj.ejcn.1602648</doi><tpages>5</tpages><oa>free_for_read</oa></addata></record> |
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| subjects | Acids Amino Acid Metabolism, Inborn Errors - blood Amino Acid Metabolism, Inborn Errors - diet therapy Amino Acid Metabolism, Inborn Errors - urine Amino acids Amino Acids - blood Amino Acids - metabolism Amino Acids - urine Aminoacid disorders Biological and medical sciences Carnitine Clinical Nutrition Conjugates Conjugation Depletion Diet diet therapy Diet, Protein-Restricted diet-related diseases dietary protein dietary protein restriction therapy Dietary Proteins - administration & dosage dietary restriction Dietary restrictions Dietary supplements Epidemiology Errors of metabolism excretion free amino acids Glycine glycine (amino acid) Humans Infant, Newborn Internal Medicine isovaleric acid isovaleric acidemia Leucine leucine restriction Liquid chromatography Mass spectrometry Mass spectroscopy Medical sciences Medicine Medicine & Public Health Metabolic Diseases Metabolites nutrient intake nutritional intervention original-article Patients Pentanoic Acids - blood Pentanoic Acids - metabolism Pentanoic Acids - urine protein supplements Proteins Public Health signs and symptoms (animals and humans) special diets Spectrometry, Mass, Electrospray Ionization - methods Treatment Outcome Urine valeric acid |
| title | Amino-acid depletion induced by abnormal amino-acid conjugation and protein restriction in isovaleric acidemia |
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