:Metabolite:OriginalMetaboliteMNXM480631245.16271fatty acyl carnitines0Q271402432-MethylbutyroylcarnitineComponent_2411Single830No-4.07NoNo86.44-7.10026.51066.43Yes4.34Methylbutyroyl-carnitine,L-2-Methylbutyrate (3-carboxy-2-hydroxypropyl)trimethyl-hydroxide ammonium inner salt,(2-Methylbutyryl)carnitine,3-[(2-Methylbutanoyl)oxy]-4-(trimethylammonio)butanoic acid,3-[(2-Methylbutanoyl)oxy]-4-(trimethylammonio)butanoate,HMDB01963,HMDB00378,HMDB0001963,Belongs to the class of organic compounds known as acyl carnitines. These are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine through an ester bond.Yes49323206426901InChIKey=IHCPDBBYTYJYIL-UHFFFAOYSA-NSolid536731023-25-3CCC(C)C(=O)OC(CC(=O)[O-])C[N+](C)(C)CInChI=1S/C12H23NO4/c1-6-9(2)12(16)17-10(7-11(14)15)8-13(3,4)5/h9-10H,6-8H2,1-5H3245.3153245.162708229detectedC12H23NO43-[(2-methylbutanoyl)oxy]-4-(trimethylazaniumyl)butanoate["2-Methylbutyroylcarnitine","2-Methylbutyrylcarnitine","2-methylbutyrylcarnitine"]["HMDB0000378","HMDB0001963","HMDB00378"]73026["ChEBI_ID,ChEBI_ID","Name"] :DiseaseAutosomal dominant polycystic kidney diseaseQ2732398 :PublicationAsymmetric dimethylarginine and lipid peroxidation products in early autosomal dominant polycystic kidney diseaseWang D, Strandgaard S, Borresen ML, Luo Z, Connors SG, Yan Q, Wilcox CS2 1018215696 doi: 10 2008 FebAm J Kidney Dis51 :PublicationGronwald W, Klein MS, Zeltner R, Schulze BD, Reinhold SW, Deutschmann M, Immervoll AK, Boger CA, Banas B, Eckardt KU, Oefner PJ 1021389975Detection of autosomal dominant polycystic kidney disease by NMR spectroscopic fingerprinting of urine 2011 Jun7911Kidney Int doi: 10 :TaxonomyOrganic compoundsKingdom :TaxonomyLipids and lipid-like moleculesSuper Class :TaxonomyClassFatty Acyls :TaxonomyFatty acid estersSub Class :TaxonomyAcyl carnitines :TaxonomyAmines :TaxonomyBranched fatty acids :TaxonomyCarbonyl compounds :TaxonomyCarboxylic acid esters :TaxonomyCarboxylic acid salts :TaxonomyCarboxylic acids :TaxonomyDicarboxylic acids and derivatives :TaxonomyHydrocarbon derivatives :TaxonomyOrganic oxides :TaxonomyOrganic salts :TaxonomyOrganopnictogen compounds :TaxonomyTetraalkylammonium salts :CelularLocationExtracellular :CelularLocationMembrane :BioSpecimenBlood :BioSpecimenUrine :TissuePlacentaQ1212935 :Publication3383426Urinary acylcarnitines in a patient with neonatal multiple acyl-CoA dehydrogenation deficiency, quantified by a carboxylic acid analyzer with a reversed-phase columnKidouchi K, Niwa T, Nohara D, Asai K, Sugiyama N, Morishita H, Kobayashi M, Wada Y 1988 Apr 293263-72Clin Chim Acta173 :Publication7970932Involvement of erythrocyte calpain in glycine- and carnitine-treated isovaleric acidemiaSalamino F, Di Lisa F, Burlina AB, Menabo R, Barbato R, De Tullio R, Siliprandi N 1994 Aug2182-6Pediatr Res36 :PublicationAbdenur JE, Chamoles NA, Guinle AE, Schenone AB, Fuertes AN624-309762597Diagnosis of isovaleric acidaemia by tandem mass spectrometry: false positive result due to pivaloylcarnitine in a newborn screening programme 1998 Aug216J Inherit Metab Dis :Publication8804338Isovaleric acidemia: response to a leucine load after three weeks of supplementation with glycine, L-carnitine, and combined glycine-carnitine therapyFries MH, Rinaldo P, Schmidt-Sommerfeld E, Jurecki E, Packman S 1996 Sep3449-52J Pediatr129 :Publication3794887Endogenous catabolism is the major source of toxic metabolites in isovaleric acidemiaMillington DS, Roe CR, Maltby DA, Inoue F 1987 Jan156-60J Pediatr110 :PublicationMinkler PE, Ingalls ST, Hoppel CL2344045High-performance liquid chromatographic separation of acylcarnitines following derivatization with 4'-bromophenacyl trifluoromethanesulfonate 1990 Feb 15129-35Anal Biochem185 :Publication6441143Urinary excretion of l-carnitine and acylcarnitines by patients with disorders of organic acid metabolism: evidence for secondary insufficiency of l-carnitineChalmers RA, Roe CR, Stacey TE, Hoppel CL 1984 Dec121325-8Pediatr Res18 :Publication6549017L-carnitine therapy in isovaleric acidemiaRoe CR, Millington DS, Maltby DA, Kahler SG, Bohan TP 1984 Dec62290-5J Clin Invest74 :PublicationRoe CR, Cederbaum SD, Roe DS, Mardach R, Galindo A, Sweetman L264-719889013Isolated isobutyryl-CoA dehydrogenase deficiency: an unrecognized defect in human valine metabolism 1998 Dec654Mol Genet Metab :Publication2062823Analysis of acylcarnitines in maternal urine for prenatal diagnosis of glutaric aciduria type 2Sakuma T, Sugiyama N, Ichiki T, Kobayashi M, Wada Y, Nohara D 1991 Feb277-82Prenat Diagn11 :PublicationMatern D, He M, Berry SA, Rinaldo P, Whitley CB, Madsen PP, van Calcar SC, Lussky RC, Andresen BS, Wolff JA, Vockley J74-812837870Prospective diagnosis of 2-methylbutyryl-CoA dehydrogenase deficiency in the Hmong population by newborn screening using tandem mass spectrometry 2003 Jul1 Pt 1Pediatrics112 :Publication2825678Pontremoli S, Melloni E, Michetti M, Sparatore B, Salamino F, Siliprandi N, Horecker BL31189-95Isovalerylcarnitine is a specific activator of calpain of human neutrophils 1987 Nov 13148Biochem Biophys Res Commun :PublicationFerrara F, Bertelli A, Falchi M16033249Evaluation of carnitine, acetylcarnitine and isovalerylcarnitine on immune function and apoptosis 2005313109-14Drugs Exp Clin Res :PublicationBene J, Komlosi K, Havasi V, Talian G, Gasztonyi B, Horvath K, Mozsik G, Hunyady B, Melegh B, Figler M110-316440427Changes of plasma fasting carnitine ester profile in patients with ulcerative colitis 2006 Jan 7121World J Gastroenterol :Publication8853559Acylcarnitine removal in a patient with acyl-CoA beta-oxidation deficiency disorder: effect of L-carnitine therapy and starvationFontaine M, Briand G, Vallee L, Ricart G, Degand P, Divry P, Vianey-Saban C, Vamecq J 1996 Aug 302109-22Clin Chim Acta252 :Publication1777996Prenatal diagnosis of isovaleric acidemia by fast atom bombardment and tandem mass spectrometryShigematsu Y, Kikawa Y, Sudo M, Kanaoka H, Fujioka M, Dan M 1991 Dec 162-3369-74Clin Chim Acta203 :PublicationMetabolomic prediction of fetal congenital heart defect in the first trimesterBahado-Singh RO, Ertl R, Mandal R, Bjorndahl TC, Syngelaki A, Han B, Dong E, Liu PB, Alpay-Savasan Z, Wishart DS, Nicolaides KH3 1024704061e1-240 2014 SepAm J Obstet Gynecol211 :PublicationZordoky BN, Sung MM, Ezekowitz J, Mandal R, Han B, Bjorndahl TC, Bouatra S, Anderson T, Oudit GY, Wishart DS, Dyck JR 1026010610Metabolomic fingerprint of heart failure with preserved ejection fraction 2015 May 26105PLoS One doi: 10 :PublicationSubstrate specificity of human carnitine acetyltransferase: Implications for fatty acid and branched-chain amino acid metabolismViolante S, Ijlst L, Ruiter J, Koster J, van Lenthe H, Duran M, de Almeida IT, Wanders RJ, Houten SM, Ventura FV6 1023485643 doi: 10 2013 JunBiochim Biophys Acta1832 :PublicationThe Metabolomic Signature of the Placenta in Spontaneous Preterm BirthElshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA3 1032033212 pii: ijms2103104321Int J Mol Sci 2020 Feb 4 :PublicationFRITZ IB13825279Action of carnitine on long chain fatty acid oxidation by liver 1959 Aug197:297-304. doi: 10.1152/ajplegacy.1959.197.2.297. 10Am J Physiol doi: 10 :Publication31920980Decreases in Circulating Concentrations of Long-Chain Acylcarnitines and Free Fatty Acids During the Glucose Tolerance Test Represent Tissue-Specific Insulin SensitivityMakarova E, Makrecka-Kuka M, Vilks K, Volska K, Sevostjanovs E, Grinberga S, Zarkova-Malkova O, Dambrova M, Liepinsh E10:870. doi: 10.3389/fendo.2019.00870. eCollection 2019.Lausanne 10 doi: 10 2019 Dec 17Front Endocrinol (Lausanne) :Publication29265114Zhang X, Li Y, Liang Y, Sun P, Wu X, Song J, Sun X, Hong M, Gao P, Deng D 2017 Nov-DecDistinguishing Intracerebral Hemorrhage from Acute Cerebral Infarction through Metabolomics doi: 106 10Rev Invest Clin69 :Publication20111019Increased levels of plasma acylcarnitines in obesity and type 2 diabetes and identification of a marker of glucolipotoxicityMihalik SJ, Goodpaster BH, Kelley DE, Chace DH, Vockley J, Toledo FG, DeLany JP18Silver Spring 10 doi: 10 2010 SepObesity (Silver Spring) :PublicationA Plasma Metabolomic Profiling of Exudative Age-Related Macular Degeneration Showing Carnosine and Mitochondrial DeficienciesChao de la Barca JM, Rondet-Courbis B, Ferre M, Muller J, Buisset A, Leruez S, Plubeau G, Mace T, Moureauzeau L, Chupin S, Tessier L, Blanchet O, Lenaers G, Procaccio V, Mirebeau-Prunier D, Simard G, Gohier P, Milea D, Reynier P3 1032120889 pii: jcm90306319J Clin Med 2020 Feb 27 :PublicationSun Y, Gao HY, Fan ZY, He Y, Yan YXMetabolomics Signatures in Type 2 Diabetes: A Systematic Review and Integrative Analysis4 1031782507 pii: 5645632 2020 Apr 1J Clin Endocrinol Metab105 :MeasurementMeasurement_228589 :Sample1Sample_35161 :ExperimentalMethodExcretionLC-PDA-MS , LC-ICP-MS , HN-APCI-MS , LC-ESI-MRM-MS , RP-HPLC-MS , HPLC-MS , LC-qTOF-MS , HPLC-qTOF-MS , UPLC-qTOF-MSExperimentalMethod_28LC-MS :UnitOtherotherUnit_138not annotated :BioSpecimenUrine, 24-hSpecimen_1Excretion :Subject69.0Meat and fish consumersFRFemale + MaleHealthySubject_229546All :PublicationA metabolomic study of biomarkers of meat and fish intakeCheung, W.; Keski-Rahkonen, P.; Assi, N.; Ferrari, P.; Freisling, H.; Rinaldi, S.; Slimani, N.; Zamora-Ros, R.; Rundle, M.; Frost, G.; Gibbons, H.; Carr, E.; Brennan, L.; Cross, A. J.; Pala, V.; Panico, S.; Sacerdote, C.; Palli, D.; Tumino, R.; Kuhn, T.; Kaaks, R.; Boeing, H.; Floegel, A.; Mancini, F.; Boutron-Ruault, M. C.; Baglietto, L.; Trichopoulou, A.; Naska, A.; Orfanos, P.; Scalbert, A.10.3945/ajcn.116.146639trueCheungtruetrue28122782InterventionAm J Clin Nutr1053Publication_10382017 :Cohorthttp://epic.iarc.fr/EPIC (European Prospective Investigation into Cancer and Nutrition)EPICObservationCohort_499126529 :MeSHPolycystic Kidney, Autosomal DominantD016891Descriptor :MeSHPlacentaD010920Descriptor :Diseasefeline polycystic kidney diseaseQ733477http://www.wikidata.org/entity/Q733477 :Diseasefeline polycystic kidney diseaseQ733477http://www.wikidata.org/entity/Q733477 :Diseasefeline polycystic kidney diseaseQ733477http://www.wikidata.org/entity/Q733477 :GeneENSG0000000871021387112185899human chromosome 1616p13.3reverse strand90086013132505310Q14914567PKD1http://www.wikidata.org/entity/Q14914567 :Tissueanterior pituitaryQ356002http://www.wikidata.org/entity/Q356002 :Tissuehttp://www.wikidata.org/entity/Q383249Q383249gastric mucosa :Tissueright coronary arteryQ429285http://www.wikidata.org/entity/Q429285 :Tissuehttp://www.wikidata.org/entity/Q707468Q707468popliteal artery :Tissueleft coronary arteryQ744391http://www.wikidata.org/entity/Q744391 :Tissuecanal of the cervixQ785307http://www.wikidata.org/entity/Q785307 :Tissuehttp://www.wikidata.org/entity/Q1978198Q1978198tibial nerve :Tissuehttp://www.wikidata.org/entity/Q2349469Q2349469ascending aorta :Tissueright uterine tubeQ66509808http://www.wikidata.org/entity/Q66509808 :Tissuehttp://www.wikidata.org/entity/Q66509809Q66509809left uterine tube :ExternalEquivalenthttp://identifiers.org/ncbigene/53105310 :MeSHBloodD001769Descriptor :MeSHUrineD014556Descriptor :MeSHBloodM0002669Concept :MeSHUrineM0022378Concept :MeSHPlacentoma, NormalM000628726Concept :MeSHPlacentaM0016933Concept PART_OF_CLADE PART_OF_CLADE PART_OF_CLADE TAKEN_FROM_SAMPLE USING_METHOD MEASURED_IN FOUND_IN TAKEN_FROM_SUBJECT CITED_IN PART_OF_COHORT CONVERTED_INTO RELATED_MESH RELATED_MESH SUBCLASS_OF SUBCLASS_OF SUBCLASS_OF ASSOCIATED_DISEASE_GENE EXPRESSED_IN EXPRESSED_IN EXPRESSED_IN EXPRESSED_IN EXPRESSED_IN EXPRESSED_IN EXPRESSED_IN EXPRESSED_IN EXPRESSED_IN EXPRESSED_IN EQUALS RELATED_MESH RELATED_MESH RELATED_MESH RELATED_MESH RELATED_MESH RELATED_MESH ASSOCIATED_DISEASE_METABOLITE CITED_IN CITED_IN18215696, CITED_IN21389975, CITED_IN CITED_IN CITED_IN CITED_IN CITED_IN CITED_IN CITED_IN CITED_IN CITED_IN CITED_IN CITED_IN CITED_IN CITED_IN CITED_IN CITED_IN CITED_IN CITED_IN CITED_IN CITED_IN CITED_IN CITED_IN CITED_IN CITED_IN CITED_IN CITED_IN MEASURED_AS PART_OF_CLADE PART_OF_CLADE PART_OF_CLADE PART_OF_CLADE PART_OF_CLADE PART_OF_CLADE PART_OF_CLADE PART_OF_CLADE PART_OF_CLADE PART_OF_CLADE PART_OF_CLADE PART_OF_CLADE PART_OF_CLADE PART_OF_CLADE LOCATED_INSIDE_CELL LOCATED_INSIDE_CELL LOCATED_IN_BIOSPECIMEN LOCATED_IN_BIOSPECIMEN LOCATED_IN_TISSUE SYNONYM_OF