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3-methylbutyrylcarnitine

Known as: isovalerylcarnitine 
National Institutes of Health

Related topics

Related topics

2 relations

Broader (1)

Carnitine
analogs & derivatives

Papers overview

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Highly Cited
2016
Highly Cited
2016
Effect of Dried Blood Spot Quality on Newborn Screening Analyte Concentrations and Recommendations for Minimum Acceptance Criteria for Sample Analysis.
BACKGROUND The analysis of dried blood spots has been used routinely for newborn screening since the early 1970s, and the number… 
Highly Cited
2009
Highly Cited
2009
Human urinary metabolomic profile of PPARalpha induced fatty acid beta-oxidation.
Activation of the peroxisome proliferator-activated receptor alpha (PPARalpha) is associated with increased fatty acid catabolism… 
Highly Cited
2000
Highly Cited
2000
A library of atmospheric pressure ionization daughter ion mass spectra based on wideband excitation in an ion trap mass spectrometer.
A searchable library of MS/MS spectra obtained using a quadrupole ion trap mass spectrometer and electrospray or atmospheric… 
Highly Cited
1996
Highly Cited
1996
Prenatal Diagnosis of Organic Acidemias Based on Amniotic Fluid Levels of Acylcarnitines
Acylcarnitines in amniotic fluid samples were analyzed for the prenatal diagnosis of propionic acidemia, methylmalonic aciduria… 
1986
1986
The response to l-carnitine and glycine therapy in isovaleric acidaemia
The profound metabolic disturbances which occur in isovaleric acidaemia are due to the intramitochondrial accumulation of… 
1986
1986
Metabolism and excretion of carnitine and acylcarnitines in the perfused rat kidney.
Highly Cited
1984
Highly Cited
1984
L-carnitine therapy in isovaleric acidemia.
Isovaleric acidemia, resulting from isovaleryl-coenzyme A dehydrogenase deficiency, is associated with marked reduction of free… 
1982
1982
Short-chain acylcarnitines of human blood and urine.
1979
1979
4-Methyl-2-oxopentanoate oxidation by rat skeletal-muscle mitochondria.
1. Oxidative decarboxylation of 4-methyl-2-oxopentanoate (2-oxoisocaproate) by mitochondria of rat skeletal muscle showed… 
Highly Cited
1970
Highly Cited
1970
Formation of branched chain acylcarnitines in mitochondria.
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