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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2022-03-07 02:49:06 UTC
HMDB IDHMDB0000756
Secondary Accession Numbers
  • HMDB0000705
  • HMDB00705
  • HMDB00756
Metabolite Identification
Common NameHexanoylcarnitine
DescriptionHexanoylcarnitine (CAS: 6418-78-6), also known as caproylcarnitine, is an acylcarnitine. Acylcarnitines are the product of the conjugation of carnitine with acyl-coenzyme A, that allows the transport of fatty acids across mitochondrial membranes. Long or medium chain fatty acids are then oxidized in the mitochondria for energy production. The ionic nature of L-carnitine causes high water solubility which decreases with increasing chain lengths of the ester group in the acylcarnitines. Therefore, the distribution of L-carnitine and acylcarnitines in various organs is defined not only by their function but also by their physicochemical properties. Hexanoylcarnitine is a medium-chain acylcarnitine present in the urine of patients with medium-chain acyl-CoA dehydrogenase deficiency (PMID: 1635814 ). Hexanoylcarnitine is also found to be associated with celiac disease and glutaric aciduria II which are both inborn errors of metabolism. Numerous disorders have been described that lead to disturbances in energy production and in intermediary metabolism, which are characterized by the production and excretion of unusual acylcarnitines. A mutation in the gene coding for carnitine-acylcarnitine translocase or the OCTN2 transporter aetiologically causes a carnitine deficiency that results in poor intestinal absorption of dietary L-carnitine, its impaired reabsorption by the kidney and, consequently, in increased urinary loss of L-carnitine. Determination of the qualitative pattern of acylcarnitines can be of diagnostic and therapeutic importance. The betaine structure of carnitine requires special analytical procedures for recording. High-performance liquid chromatography (HPLC) permits screening for free and total carnitine, as well as complete quantitative acylcarnitine determination, including the long-chain acylcarnitine profile (PMID: 17508264 , Monatshefte fuer Chemie (2005), 136(8), 1279-1291., Int J Mass Spectrom. 1999;188:39-52.).
Structure
Data?1614284811
Synonyms
ValueSource
(R)-CaproylcarnitineChEBI
(R)-HexanoylcarnitineChEBI
Hexanoyl-L-carnitineChEBI
L-HexanoylcarnitineChEBI
O-Hexanoyl-(R)-carnitineChEBI
(R)-3-Carboxy-N,N,N-trimethyl-2-[(1-oxohexyl)oxy]-1-propanaminium inner saltHMDB
HexanoateHMDB
Hexanoic acidHMDB
Hexanoic acid ester with L-(3-carboxy-2-hydroxypropyl)trimethylammonium hydroxide inner saltHMDB
L-Carnitine hexanoyl esterHMDB
L-HexanoateHMDB
L-Hexanoic acidHMDB
L-Hexanoic acid ester with (3-carboxy-2-hydroxypropyl)trimethylammonium hydroxide inner saltHMDB
C6 CarnitineHMDB
HexanoylcarnitineHMDB
Chemical FormulaC13H26NO4
Average Molecular Weight260.3498
Monoisotopic Molecular Weight260.186183325
IUPAC Name(3R)-3-(hexanoyloxy)-4-(trimethylazaniumyl)butanoate
Traditional NameL-carnitine hexanoyl ester
CAS Registry Number22671-29-0
SMILES
CCCCCC(=O)O[C@H](CC(O)=O)C[N+](C)(C)C
InChI Identifier
InChI=1S/C13H25NO4/c1-5-6-7-8-13(17)18-11(9-12(15)16)10-14(2,3)4/h11H,5-10H2,1-4H3/p+1/t11-/m1/s1
InChI KeyVVPRQWTYSNDTEA-LLVKDONJSA-O
Chemical Taxonomy
Description 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.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassFatty Acyls
Sub ClassFatty acid esters
Direct ParentAcyl carnitines
Alternative Parents
Substituents
  • Acyl-carnitine
  • Dicarboxylic acid or derivatives
  • Tetraalkylammonium salt
  • Quaternary ammonium salt
  • Carboxylic acid ester
  • Carboxylic acid salt
  • Carboxylic acid derivative
  • Carboxylic acid
  • Organic nitrogen compound
  • Organooxygen compound
  • Organonitrogen compound
  • Organic salt
  • Hydrocarbon derivative
  • Organic oxide
  • Organopnictogen compound
  • Organic oxygen compound
  • Carbonyl group
  • Amine
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect
Disposition
Process
Role
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
PropertyValueSource
Water Solubility0.0043 g/LALOGPS
logP-1.7ALOGPS
logP-2.4ChemAxon
logS-4.9ALOGPS
pKa (Strongest Acidic)4.22ChemAxon
pKa (Strongest Basic)-7.1ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count0ChemAxon
Polar Surface Area66.43 ŲChemAxon
Rotatable Bond Count10ChemAxon
Refractivity91.07 m³·mol⁻¹ChemAxon
Polarizability28.79 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DeepCCS[M+H]+164.80230932474
DeepCCS[M-H]-162.44430932474
DeepCCS[M-2H]-195.32830932474
DeepCCS[M+Na]+170.89630932474

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
HexanoylcarnitineCCCCCC(=O)O[C@H](CC(O)=O)C[N+](C)(C)C2699.0Standard polar33892256
HexanoylcarnitineCCCCCC(=O)O[C@H](CC(O)=O)C[N+](C)(C)C1539.5Standard non polar33892256
HexanoylcarnitineCCCCCC(=O)O[C@H](CC(O)=O)C[N+](C)(C)C1772.5Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
Hexanoylcarnitine,1TMS,isomer #1CCCCCC(=O)O[C@H](CC(=O)O[Si](C)(C)C)C[N+](C)(C)C1808.0Semi standard non polar33892256
Hexanoylcarnitine,1TBDMS,isomer #1CCCCCC(=O)O[C@H](CC(=O)O[Si](C)(C)C(C)(C)C)C[N+](C)(C)C2035.1Semi standard non polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Predicted GC-MSPredicted GC-MS Spectrum - Hexanoylcarnitine GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Hexanoylcarnitine GC-MS (TMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Hexanoylcarnitine GC-MS (TBDMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - Hexanoylcarnitine Quattro_QQQ 10V, Positive-QTOF (Annotated)splash10-03dr-6090000000-c19afe5acca5a4b0f82f2012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Hexanoylcarnitine Quattro_QQQ 25V, Positive-QTOF (Annotated)splash10-000i-9000000000-8d30a3ad5fce9bf004f72012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Hexanoylcarnitine Quattro_QQQ 40V, Positive-QTOF (Annotated)splash10-000i-9000000000-a38bcb0bb25abdcaaa7c2012-07-24HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Hexanoylcarnitine 10V, Positive-QTOFsplash10-03di-0090000000-5c4e7ecd71d0ed800ae62021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Hexanoylcarnitine 20V, Positive-QTOFsplash10-01p9-9050000000-f92580b9643caf52768e2021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Hexanoylcarnitine 40V, Positive-QTOFsplash10-000i-9000000000-e9262cbaff8cb4ad0ba62021-09-22Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Predicted 1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Experimental 2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, CD3OD, experimental)2012-12-05Wishart LabView Spectrum
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biospecimen Locations
  • Blood
  • Feces
  • Urine
Tissue Locations
  • Placenta
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.080 +/- 0.006 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.020-0.130 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified<0.12 uMAdolescent (13-18 years old)MaleNormal details
BloodDetected and Quantified0.06 +/- 0.02 uMAdult (>18 years old)BothNormal details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.020-0.130 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified0.031 (0.015-0.053) uMChildren (1-13 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedNormal details
FecesDetected and Quantified0.23 +/- 0.13 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
FecesDetected and Quantified0.18 +/- 0.08 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0.02-0.05 umol/mmol creatinineNewborn (0-30 days old)BothNormal details
UrineDetected and Quantified0.03 +/- 0.01 umol/mmol creatinineNewborn (0-30 days old)FemaleNormal details
UrineDetected and Quantified0.03 +/- 0.01 umol/mmol creatinineNewborn (0-30 days old)MaleNormal details
UrineDetected and Quantified0.05 (0.03-0.07) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.060 +/- 0.004 uMAdult (>18 years old)BothCeliac disease details
BloodDetected and Quantified0.18 uMAdolescent (13-18 years old)Male
Glutaric aciduria II
details
BloodDetected and Quantified0.31 uMAdolescent (13-18 years old)MaleGlutaric aciduria II details
BloodDetected and Quantified0.051 +/- 0.016 uMChildren (1-13 years old)Both
Obesity
    • Metabolomics reve...
details
BloodDetected and Quantified0.058 +/- 0.025 uMChildren (1-13 years old)Both
Obesity
    • Metabolomics reve...
details
BloodDetected and Quantified0.001 (0.0-0.004) uMAdult (>18 years old)BothVery long-chain acyl-CoA dehydrogenase deficiency (vLCAD) details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedCrohns disease details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedUlcerative colitis details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedUnclassified IBD details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
Associated Disorders and Diseases
Disease References
Celiac disease
  1. Bene J, Komlosi K, Gasztonyi B, Juhasz M, Tulassay Z, Melegh B: Plasma carnitine ester profile in adult celiac disease patients maintained on long-term gluten free diet. World J Gastroenterol. 2005 Nov 14;11(42):6671-5. [PubMed:16425363 ]
Very Long Chain Acyl-CoA Dehydrogenase Deficiency
  1. Costa CG, Struys EA, Bootsma A, ten Brink HJ, Dorland L, Tavares de Almeida I, Duran M, Jakobs C: Quantitative analysis of plasma acylcarnitines using gas chromatography chemical ionization mass fragmentography. J Lipid Res. 1997 Jan;38(1):173-82. [PubMed:9034211 ]
Glutaric aciduria II
  1. Prasad M, Hussain S: Glutaric aciduria type II presenting as myopathy and rhabdomyolysis in a teenager. J Child Neurol. 2015 Jan;30(1):96-9. doi: 10.1177/0883073813516676. Epub 2014 Jan 21. [PubMed:24453145 ]
Obesity
  1. Simone Wahl, Christina Holzapfel, Zhonghao Yu, Michaela Breier, Ivan Kondofersky, Christiane Fuchs, Paula Singmann, Cornelia Prehn, Jerzy Adamski, Harald Grallert, Thomas Illig, Rui Wang-Sattler, Thomas Reinehr (2013). Metabolomics reveals determinants of weight loss during lifestyle intervention in obese children. Metabolomics.
Crohn's disease
  1. Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Ulcerative colitis
  1. Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Colorectal cancer
  1. Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
Associated OMIM IDs
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB022226
KNApSAcK IDC00052313
Chemspider ID2497478
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN ID3548
PubChem Compound3246938
PDB IDNot Available
ChEBI ID84834
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDMDB00000244
Good Scents IDNot Available
References
Synthesis ReferenceNakanishi, Toyofumi; Shimizu, Akira; Arimoto, Masao; Kanai, Michiko. Synthesis of acylcarnitines for differential diagnosis of metabolic disorders. Nippon Iyo Masu Supekutoru Gakkai Koenshu (1993), 18 129-32.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Ziadeh R, Hoffman EP, Finegold DN, Hoop RC, Brackett JC, Strauss AW, Naylor EW: Medium chain acyl-CoA dehydrogenase deficiency in Pennsylvania: neonatal screening shows high incidence and unexpected mutation frequencies. Pediatr Res. 1995 May;37(5):675-8. [PubMed:7603790 ]
  2. Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762. [PubMed:19212411 ]
  3. Minkler PE, Hoppel CL: Quantification of free carnitine, individual short- and medium-chain acylcarnitines, and total carnitine in plasma by high-performance liquid chromatography. Anal Biochem. 1993 Aug 1;212(2):510-8. [PubMed:8214594 ]
  4. Chalmers RA, Roe CR, Stacey TE, Hoppel CL: Urinary excretion of l-carnitine and acylcarnitines by patients with disorders of organic acid metabolism: evidence for secondary insufficiency of l-carnitine. Pediatr Res. 1984 Dec;18(12):1325-8. [PubMed:6441143 ]
  5. Fontaine M, Briand G, Vallee L, Ricart G, Degand P, Divry P, Vianey-Saban C, Vamecq J: Acylcarnitine removal in a patient with acyl-CoA beta-oxidation deficiency disorder: effect of L-carnitine therapy and starvation. Clin Chim Acta. 1996 Aug 30;252(2):109-22. [PubMed:8853559 ]
  6. Lee PJ, Harrison EL, Jones MG, Jones S, Leonard JV, Chalmers RA: L-carnitine and exercise tolerance in medium-chain acyl-coenzyme A dehydrogenase (MCAD) deficiency: a pilot study. J Inherit Metab Dis. 2005;28(2):141-52. [PubMed:15877203 ]
  7. Tyni T, Pourfarzam M, Turnbull DM: Analysis of mitochondrial fatty acid oxidation intermediates by tandem mass spectrometry from intact mitochondria prepared from homogenates of cultured fibroblasts, skeletal muscle cells, and fresh muscle. Pediatr Res. 2002 Jul;52(1):64-70. [PubMed:12084849 ]
  8. Caksen H, Tuncer O, Kirimi E, Fryns JP, Uner A, Unal O, Cinal A, Odabas D: Report of two Turkish infants with Norman-Roberts syndrome. Genet Couns. 2004;15(1):9-17. [PubMed:15083694 ]
  9. Schmidt-Sommerfeld E, Penn D, Rinaldo P, Kossak D, Li BU, Huang ZH, Gage DA: Urinary medium-chain acylcarnitines in medium-chain acyl-CoA dehydrogenase deficiency, medium-chain triglyceride feeding and valproic acid therapy: sensitivity and specificity of the radioisotopic exchange/high performance liquid chromatography method. Pediatr Res. 1992 Jun;31(6):545-51. [PubMed:1635814 ]
  10. Shigematsu Y, Hata I, Nakai A, Kikawa Y, Sudo M, Tanaka Y, Yamaguchi S, Jakobs C: Prenatal diagnosis of organic acidemias based on amniotic fluid levels of acylcarnitines. Pediatr Res. 1996 Apr;39(4 Pt 1):680-4. [PubMed:8848345 ]
  11. Schmidt-Sommerfeld E, Penn D, Duran M, Bennett MJ, Santer R, Stanley CA: Detection of inborn errors of fatty acid oxidation from acylcarnitine analysis of plasma and blood spots with the radioisotopic exchange-high-performance liquid chromatographic method. J Pediatr. 1993 May;122(5 Pt 1):708-14. [PubMed:8496747 ]
  12. Bene J, Komlosi K, Gasztonyi B, Juhasz M, Tulassay Z, Melegh B: Plasma carnitine ester profile in adult celiac disease patients maintained on long-term gluten free diet. World J Gastroenterol. 2005 Nov 14;11(42):6671-5. [PubMed:16425363 ]
  13. Bennett MJ, Coates PM, Hale DE, Millington DS, Pollitt RJ, Rinaldo P, Roe CR, Tanaka K: Analysis of abnormal urinary metabolites in the newborn period in medium-chain acyl-CoA dehydrogenase deficiency. J Inherit Metab Dis. 1990;13(5):707-15. [PubMed:2246856 ]
  14. Vallee L, Fontaine M, Nuyts JP, Ricart G, Krivosic I, Divry P, Vianey-Saban C, Lhermitte M, Vamecq J: Stroke, hemiparesis and deficient mitochondrial beta-oxidation. Eur J Pediatr. 1994 Aug;153(8):598-603. [PubMed:7957409 ]
  15. Schmidt-Sommerfeld E, Penn D, Kerner J, Bieber LL, Rossi TM, Lebenthal E: Quantitation of urinary carnitine esters in a patient with medium-chain acyl-coenzyme A dehydrogenase deficiency: effect of metabolic state and L-carnitine therapy. J Pediatr. 1989 Oct;115(4):577-82. [PubMed:2795349 ]
  16. Minkler PE, Hoppel CL: Quantification of carnitine and specific acylcarnitines by high-performance liquid chromatography: application to normal human urine and urine from patients with methylmalonic aciduria, isovaleric acidemia or medium-chain acyl-CoA dehydrogenase deficiency. J Chromatogr. 1993 Apr 2;613(2):203-21. [PubMed:8491807 ]
  17. Pierre G, Macdonald A, Gray G, Hendriksz C, Preece MA, Chakrapani A: Prospective treatment in carnitine-acylcarnitine translocase deficiency. J Inherit Metab Dis. 2007 Oct;30(5):815. Epub 2007 May 12. [PubMed:17508264 ]
  18. Brunk E, Sahoo S, Zielinski DC, Altunkaya A, Drager A, Mih N, Gatto F, Nilsson A, Preciat Gonzalez GA, Aurich MK, Prlic A, Sastry A, Danielsdottir AD, Heinken A, Noronha A, Rose PW, Burley SK, Fleming RMT, Nielsen J, Thiele I, Palsson BO: Recon3D enables a three-dimensional view of gene variation in human metabolism. Nat Biotechnol. 2018 Mar;36(3):272-281. doi: 10.1038/nbt.4072. Epub 2018 Feb 19. [PubMed:29457794 ]
  19. Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212 ]