3-butanoyloxy-4-trimethylammonio-butanoate
| Title | Journal |
|---|---|
| A small molecule inhibitor of mutant IDH2 rescues cardiomyopathy in a D-2-hydroxyglutaric aciduria type II mouse model. | Journal of inherited metabolic disease 20160101 |
| Biochemical, molecular, and clinical characteristics of children with short chain acyl-CoA dehydrogenase deficiency detected by newborn screening in California. | Molecular genetics and metabolism 20120501 |
| Rapid determination of C4-acylcarnitine and C5-acylcarnitine isomers in plasma and dried blood spots by UPLC-MS/MS as a second tier test following flow-injection MS/MS acylcarnitine profile analysis. | Molecular genetics and metabolism 20100901 |
| Flavin adenine dinucleotide status and the effects of high-dose riboflavin treatment in short-chain acyl-CoA dehydrogenase deficiency. | Pediatric research 20100301 |
| Biochemical correction of short-chain acyl-coenzyme A dehydrogenase deficiency after portal vein injection of rAAV8-SCAD. | Human gene therapy 20080601 |
| Transport of butyryl-L-carnitine, a potential prodrug, via the carnitine transporter OCTN2 and the amino acid transporter ATB(0,+). | American journal of physiology. Gastrointestinal and liver physiology 20071101 |
| Persistent increase of plasma butyryl/isobutyrylcarnitine concentrations as marker of SCAD defect and ethylmalonic encephalopathy. | Journal of inherited metabolic disease 20061001 |
| Clinical, biochemical, and genetic heterogeneity in short-chain acyl-coenzyme A dehydrogenase deficiency. | JAMA 20060823 |
| Monitoring of ketogenic diet for carnitine metabolites by subcutaneous microdialysis. | Pediatric research 20060701 |
| Systemic correction of a fatty acid oxidation defect by intramuscular injection of a recombinant adeno-associated virus vector. | Human gene therapy 20060101 |
| Acyl-CoA dehydrogenase deficiency: varieties with neurological involvement. | Developmental medicine and child neurology 20050301 |
| Filter paper cards contaminated with EMLA cream produce artefacts on acylcarnitine analysis. | Journal of inherited metabolic disease 20040101 |
| A comparison of in vitro acylcarnitine profiling methods for the diagnosis of classical and variant short chain acyl-CoA dehydrogenase deficiency. | Clinica chimica acta; international journal of clinical chemistry 20031101 |
| Rare disorders of metabolism with elevated butyryl- and isobutyryl-carnitine detected by tandem mass spectrometry newborn screening. | Pediatric research 20030801 |
| Ester synthesis from trimethylammonium alcohols in dry organic media catalyzed by immobilized Candida antarctica lipase B. | Biotechnology and bioengineering 20030505 |
| The frequency of short-chain acyl-CoA dehydrogenase gene variants in the US population and correlation with the C(4)-acylcarnitine concentration in newborn blood spots. | Molecular genetics and metabolism 20030401 |
| 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. | Pediatric research 20020701 |
| A polymeric microfluidic chip for CE/MS determination of small molecules. | Analytical chemistry 20010501 |