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Inhibition of the electron transport chain and creatine kinase activity by ethylmalonic acid in human skeletal muscle.Alethea G Barschak et al.Metabolic brain disease, 21(1), 11-19 (2006-06-15) The ACADS gene variation spectrum in 114 patients with short-chain acyl-CoA dehydrogenase (SCAD) deficiency is dominated by missense variations leading to protein misfolding at the cellular level.Christina B Pedersen et al.Human genetics, 124(1), 43-56 (2008-06-05) Ethylmalonic encephalopathy-report of two cases.Lada Cindro Heberle et al.Brain & development, 28(5), 329-331 (2005-12-27) Clinical, biochemical, and genetic heterogeneity in short-chain acyl-coenzyme A dehydrogenase deficiency.Bianca T van Maldegem et al.JAMA, 296(8), 943-952 (2006-08-24) Riboflavin and riboflavin-derived cofactors in adolescent girls with anorexia nervosa.C D Capo-chichi et al.The American journal of clinical nutrition, 69(4), 672-678 (1999-04-10) Ethylmalonic acid impairs brain mitochondrial succinate and malate transport.Alexandre Umpierrez Amaral et al.Molecular genetics and metabolism, 105(1), 84-90 (2011-12-03) Cannabinoid receptor antagonist-induced striated muscle toxicity and ethylmalonic-adipic aciduria in beagle dogs.Lindsay Tomlinson et al.Toxicological sciences : an official journal of the Society of Toxicology, 129(2), 268-279 (2012-07-24) Detection of autosomal dominant polycystic kidney disease by NMR spectroscopic fingerprinting of urine.Wolfram Gronwald et al.Kidney international, 79(11), 1244-1253 (2011-03-11) Metabolism of cortisol in anorexia nervosa.H Vierhapper et al.Acta endocrinologica, 122(6), 753-758 (1990-06-01) Silent increase of urinary ethylmalonic acid is an indicator of nonspecific brain dysfunction.Raffaella Zannolli et al.NMR in biomedicine, 23(4), 353-358 (2010-02-27) Age-related reference values for urinary organic acids in a healthy Turkish pediatric population.F Guneral et al.Clinical chemistry, 40(6), 862-866 (1994-06-01) Chronic early postnatal administration of ethylmalonic acid to rats causes behavioral deficit.Patrícia Fernanda Schuck et al.Behavioural brain research, 197(2), 364-370 (2008-10-28) Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease.J D Shoemaker et al.Journal of chromatography, 562(1-2), 125-138 (1991-01-02) Hypoglycaemia and elevated urine ethylmalonic acid in a child homozygous for the short-chain acyl-CoA dehydrogenase 625G > A gene variation.N H Birkebaek et al.Acta paediatrica (Oslo, Norway : 1992), 91(4), 480-482 (2002-06-14) Antioxidant dysfunction: potential risk for neurotoxicity in ethylmalonic aciduria.Christina B Pedersen et al.Journal of inherited metabolic disease, 33(3), 211-222 (2010-05-06) Loss of ETHE1, a mitochondrial dioxygenase, causes fatal sulfide toxicity in ethylmalonic encephalopathy.Valeria Tiranti et al.Nature medicine, 15(2), 200-205 (2009-01-13) Ethylmalonic encephalopathy associated with crescentic glomerulonephritis.Imad Dweikat et al.Metabolic brain disease, 27(4), 613-616 (2012-05-16) Quantitative analysis for organic acids in biological samples: batch isolation followed by gas chromatographic-mass spectrometric analysis.G Hoffmann et al.Clinical chemistry, 35(4), 587-595 (1989-04-01) Flavin adenine dinucleotide status and the effects of high-dose riboflavin treatment in short-chain acyl-CoA dehydrogenase deficiency.Bianca T van Maldegem et al.Pediatric research, 67(3), 304-308 (2009-12-03) Effect of in vivo administration of ethylmalonic acid on energy metabolism in rat tissues.Gustavo da C Ferreira et al.Metabolic brain disease, 21(1), 29-39 (2006-06-15) Decreased oxidative phosphorylation and PGAM deficiency in horses suffering from atypical myopathy associated with acquired MADD.C M Westermann et al.Molecular genetics and metabolism, 104(3), 273-278 (2011-08-17) Ethylmalonic acid modulates Na+, K(+)-ATPase activity and mRNA levels in rat cerebral cortex.Patrícia Fernanda Schuck et al.Synapse (New York, N.Y.), 67(3), 111-117 (2012-11-20) Combined treatment with oral metronidazole and N-acetylcysteine is effective in ethylmalonic encephalopathy.Carlo Viscomi et al.Nature medicine, 16(8), 869-871 (2010-07-27) Single-nucleotide variations in the genes encoding the mitochondrial Hsp60/Hsp10 chaperone system and their disease-causing potential.Peter Bross et al.Journal of human genetics, 52(1), 56-65 (2006-10-31) Cardiomyopathy and hypotonia in a 5-month-old infant with malonyl-coa decarboxylase deficiency: potential for preclinical diagnosis with expanded newborn screening.C Ficicioglu et al.Pediatric cardiology, 26(6), 881-883 (2005-08-04) Promotion of lipid and protein oxidative damage in rat brain by ethylmalonic acid.Patrícia Fernanda Schuck et al.Neurochemical research, 35(2), 298-305 (2009-09-17) The role of methionine in ethylmalonic encephalopathy with petechiae.Karen A McGowan et al.Archives of neurology, 61(4), 570-574 (2004-04-21) A case of ethylmalonic encephalopathy with atypical clinical and biochemical presentation.Maja Di Rocco et al.Molecular genetics and metabolism, 89(4), 395-397 (2006-07-11) Ethylmalonic encephalopathy: clinical and biochemical observations.D I Zafeiriou et al.Neuropediatrics, 38(2), 78-82 (2007-08-23) Clinical and biochemical characterization of short-chain acyl-coenzyme A dehydrogenase deficiency.A Bhala et al.The Journal of pediatrics, 126(6), 910-915 (1995-06-01) Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression.Arun Sreekumar et al.Nature, 457(7231), 910-914 (2009-02-13) Severe infantile hypotonia with ethylmalonic aciduria: case report.Cetin Okuyaz et al.Journal of child neurology, 23(6), 703-705 (2008-06-10) Short-chain Acyl-CoA dehydrogenase deficiency: studies in a large family adding to the complexity of the disorder.Levinus A Bok et al.Pediatrics, 112(5), 1152-1155 (2003-11-05) Tandem mass spectrometric determination of malonylcarnitine: diagnosis and neonatal screening of malonyl-CoA decarboxylase deficiency.René Santer et al.Clinical chemistry, 49(4), 660-662 (2003-03-26) Ethylmalonic encephalopathy is caused by mutations in ETHE1, a gene encoding a mitochondrial matrix protein.Valeria Tiranti et al.American journal of human genetics, 74(2), 239-252 (2004-01-21) Clinical, biochemical, magnetic resonance imaging (MRI) and proton magnetic resonance spectroscopy (1H MRS) findings in a fourth case of combined D- and L-2 hydroxyglutaric aciduria.M-H Read et al.Journal of inherited metabolic disease, 28(6), 1149-1150 (2006-01-26) Biochemical, molecular, and clinical characteristics of children with short chain acyl-CoA dehydrogenase deficiency detected by newborn screening in California.Natalie M Gallant et al.Molecular genetics and metabolism, 106(1), 55-61 (2012-03-20) Concentrations of riboflavin and related organic acids in children with protein-energy malnutrition.C D Capo-Chichi et al.The American journal of clinical nutrition, 71(4), 978-986 (2000-03-25) Identification of ethylmalonic acid in urine of two patients with the vomitting sickness of Jamaica.K Tanaka et al.Clinica chimica acta; international journal of clinical chemistry, 69(1), 105-112 (1976-05-17) Persistent increase of plasma butyryl/isobutyrylcarnitine concentrations as marker of SCAD defect and ethylmalonic encephalopathy.B Merinero et al.Journal of inherited metabolic disease, 29(5), 685-685 (2006-08-15) Lessons to learn from rare inborn errors of metabolism.B Plecko Neuropediatrics, 38(2), 59-60 (2007-08-23)

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