InChI=1S/C24H47NO4/c1-5-6-7-8-9-10-11-12-13-14-15-16-17-18-19-24(28)29-22(20-23(26)27)21-25(2,3)4/h22H,5-21H2,1-4H3 |
DWSFAVOTORHAAL-UHFFFAOYSA-N |
C(OC(CCCCCCCCCCCCCCCC)=O)(C[N+](C)(C)C)CC(=O)[O-] |
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rat metabolite
Any mammalian metabolite produced during a metabolic reaction in rat (Rattus norvegicus).
human metabolite
Any mammalian metabolite produced during a metabolic reaction in humans (Homo sapiens).
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biomarker
A substance used as an indicator of a biological state.
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View more via ChEBI Ontology
3-(heptadecanoyloxy)-4-(trimethylazaniumyl)butanoate
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3-Carboxy-N,N,N-trimethyl-2-[(1-oxoheptadecyl)oxy]-1-propanaminium inner salt
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HMDB
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C17 carnitine
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ChEBI
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Carnitine heptadecanoate
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HMDB
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Heptadecanoyl carnitine
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HMDB
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heptadecanoylcarnitine
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ChEBI
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Liu J, Chen XX, Li XW, Fu W, Zhang WQ (2016) Metabolomic Research on Newborn Infants With Intrauterine Growth Restriction. Medicine 95, e3564 [PubMed:27124067] [show Abstract] To compare differences in metabolites between newborns with intrauterine growth restriction (IUGR) and those who are appropriate for gestational age (AGA) in order to understand the changes in metabolites of newborns with IUGR and to explore the possible metabolic mechanism of tissue and organ damages in patients with IUGR, with the ultimate goal of providing the basis for clinical intervention.A total of 60 newborns with IUGR and 60 AGA newborns who were hospitalized in the neonatal intensive care unit of our hospital between January 2011 and December 2015 and who underwent metabolic disease screening were enrolled in this study. The differences in 21 amino acids and 55 carnitines in peripheral blood, as well as changes in the ratios of free carnitine and acylcarnitine to total carnitine, were compared.Metabolites, particularly alanine, homocysteine, leucine, methionine, ornithine, serine, tyrosine, isovaleryl carnitine, and eicosenoyl carnitine, differed according to newborns' birth weight (<3rd percentile, 3rd-5th percentiles, 5th-10th percentiles, and 10th-90th percentiles), with those with lower birth weight showing the greater difference (P < 0.05). Metabolites also differed by gestational age, and the differences observed were mainly as follows: preterm and full-term newborns showed differences in metabolites, mainly in alanine, proline, cerotoyl carnitine, and tetradecanedioyl carnitine (P < 0.05); preterm and full-term AGA newborns showed differences in metabolites, mainly in alanine, glutamine, homocysteine, pipecolic acid, proline, heptanoyl carnitine, and sebacoyl carnitine (P < 0.05); and preterm and full-term newborns with IUGR showed differences in metabolites, mainly in arginine, glutamic acid, homocysteine, histidine, leucine, isoleucine, ornithine, serine, threonine, tryptophan, valine, heptanoyl carnitine, decanoyl carnitine, linoleyl carnitine, methylmalonyl carnitine, glutarylcarnitine, sebacoyl carnitine, hydroxyacetyl carnitine, and hydroxyhexadecancenyl carnitine (P < 0.05). Among newborns with IUGR, metabolites differed among males and females, mainly in aspartic acid, glutamic acid, and hexacosenoic acid (P < 0.05). Birth weight had no significant effects on free carnitine concentration or on the ratios of free carnitine and acylcarnitine to total carnitine (P < 0.05).IUGR infants exhibit significant abnormalities in amino acid and acylcarnitine metabolism, especially those with birth weight below the third percentile. With increasing birth weight, amino acids and acylcarnitines showed compensatory increases or reductions, and when birth weight reached the 10th percentile, the newborns with IUGR resembled the AGA newborns. | Malvagia S, Haynes CA, Grisotto L, Ombrone D, Funghini S, Moretti E, McGreevy KS, Biggeri A, Guerrini R, Yahyaoui R, Garg U, Seeterlin M, Chace D, De Jesus VR, la Marca G (2015) Heptadecanoylcarnitine (C17) a novel candidate biomarker for newborn screening of propionic and methylmalonic acidemias. Clinica chimica acta; international journal of clinical chemistry 450, 342-348 [PubMed:26368264] [show Abstract]
Background3-Hydroxypalmitoleoyl-carnitine (C16:1-OH) has recently been reported to be elevated in acylcarnitine profiles of patients with propionic acidemia (PA) or methylmalonic acidemia (MMA) during expanded newborn screening (NBS). High levels of C16:1-OH, combined with other hydroxylated long chain acylcarnitines are related to long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD) and trifunctional protein (TFP) deficiency.MethodsThe acylcarnitine profile of two LCHADD patients was evaluated using liquid chromatography-tandem mass spectrometric method. A specific retention time was determined for each hydroxylated long chain acylcarnitine. The same method was applied to some neonatal dried blood spots (DBSs) from PA and MMA patients presenting abnormal C16:1-OH concentrations.ResultsThe retention time of the peak corresponding to C16:1-OH in LCHADD patients differed from those in MMA and PA patients. Heptadecanoylcarnitine (C17) has been identified as the novel biomarker specific for PA and MMA patients through high resolution mass spectrometry (Orbitrap) experiments. We found that 21 out of 23 neonates (22 MMA, and 1PA) diagnosed through the Tuscany region NBS program exhibited significantly higher levels of C17 compared to controls. Twenty-three maternal deficiency (21 vitamin B12 deficiency, 1 homocystinuria and 1 gastrin deficiency) samples and 82 false positive for elevated propionylcarnitine (C3) were also analyzed.ConclusionsWe have characterized a novel biomarker able to detect propionate disorders during expanded newborn screening (NBS). The use of this new biomarker may improve the analytical performances of NBS programs especially in laboratories where second tier tests are not performed. | Minkler PE, Stoll MS, Ingalls ST, Yang S, Kerner J, Hoppel CL (2008) Quantification of carnitine and acylcarnitines in biological matrices by HPLC electrospray ionization-mass spectrometry. Clinical chemistry 54, 1451-1462 [PubMed:18678604] [show Abstract]
BackgroundAnalysis of carnitine and acylcarnitines by tandem mass spectrometry (MS/MS) has limitations. First, preparation of butyl esters partially hydrolyzes acylcarnitines. Second, isobaric nonacylcarnitine compounds yield false-positive results in acylcarnitine tests. Third, acylcarnitine constitutional isomers cannot be distinguished.MethodsCarnitine and acylcarnitines were isolated by ion-exchange solid-phase extraction, derivatized with pentafluorophenacyl trifluoromethanesulfonate, separated by HPLC, and detected with an ion trap mass spectrometer. Carnitine was quantified with d(3)-carnitine as the internal standard. Acylcarnitines were quantified with 42 synthesized calibrators. The internal standards used were d(6)-acetyl-, d(3)-propionyl-, undecanoyl-, undecanedioyl-, and heptadecanoylcarnitine.ResultsExample recoveries [mean (SD)] were 69.4% (3.9%) for total carnitine, 83.1% (5.9%) for free carnitine, 102.2% (9.8%) for acetylcarnitine, and 107.2% (8.9%) for palmitoylcarnitine. Example imprecision results [mean (SD)] within runs (n = 6) and between runs (n = 18) were, respectively: total carnitine, 58.0 (0.9) and 57.4 (1.7) micromol/L; free carnitine, 44.6 (1.5) and 44.3 (1.2) micromol/L; acetylcarnitine, 7.74 (0.51) and 7.85 (0.69) micromol/L; and palmitoylcarnitine, 0.12 (0.01) and 0.11 (0.02) micromol/L. Standard-addition slopes and linear regression coefficients were 1.00 and 0.9998, respectively, for total carnitine added to plasma, 0.99 and 0.9997 for free carnitine added to plasma, 1.04 and 0.9972 for octanoylcarnitine added to skeletal muscle, and 1.05 and 0.9913 for palmitoylcarnitine added to skeletal muscle. Reference intervals for plasma, urine, and skeletal muscle are provided.ConclusionsThis method for analysis of carnitine and acylcarnitines overcomes the observed limitations of MS/MS methods. | Minkler PE, Kerner J, North KN, Hoppel CL (2005) Quantitation of long-chain acylcarnitines by HPLC/fluorescence detection: application to plasma and tissue specimens from patients with carnitine palmitoyltransferase-II deficiency. Clinica chimica acta; international journal of clinical chemistry 352, 81-92 [PubMed:15653102] [show Abstract]
BackgroundCarnitine palmitoyltransferase-II deficiency (CPT-II deficiency) is a rare disorder of lipid metabolism, in which the accumulation of long-chain acylcarnitines is a diagnostic marker. HPLC with fluorescence detection is an attractive analysis method due to its favorable combination of sensitivity, specificity, ease of analysis and minimal capital equipment costs.MethodsLong-chain acylcarnitines were isolated from tissue homogenates (0.5-2 mg wet weight) or plasma (50 microl) using silica gel columns and derivatized with 2-(2,3-naphthalimino)ethyl trifluoromethanesulfonate. Quantitation was by HPLC and fluorescence detection with standard curves (0.0-5.0 nmol/ml) for myristoyl-, palmitoleoyl-, palmitoyl-, oleoyl- and stearoylcarnitine using heptadecanoylcarnitine as the internal standard.ResultsSignificantly greater amounts of long-chain acylcarnitines were quantified in patients with CPT-II deficiency when compared to controls; e.g. (nmol/ml in patient plasma, controls mean+/-standard deviation): myristoylcarnitine (0.3, not detectable), palmitoleoylcarnitine (0.5, 0.1+/-0.1), palmitoylcarnitine (0.9, 0.1+/-0.0), oleoylcarnitine (3.0, 0.2+/-0.1), stearoylcarnitine (0.4, not detectable).ConclusionsThis method can be used to quantitate long-chain acylcarnitines, illustrating their accumulation in CPT-II deficiency. The analysis was accomplished using inexpensive and widely available instrumentation and is appropriate for research investigators who require precise quantitation of long-chain acylcarnitines in complex biological samples. |
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