Disorders of mitochondrial fatty acyl-CoA beta-oxidation (Q28139723)

Europe PubMed Central

title

Disorders of mitochondrial fatty acyl-CoA beta-oxidation (English)

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R J Wanders

1

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P Vreken

2

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M E den Boer

3

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F A Wijburg

4

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A H van Gennip

5

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L IJlst

Journal of Inherited Metabolic Disease

6

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language of work or name

English

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publication date

June 1999

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published in

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volume

22

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issue

4

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page(s)

442-87

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cites work

Sudden infant death and long-chain 3-hydroxyacyl-CoA dehydrogenase

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12 December 2020

1 reference

12 December 2020

Short-chain acyl-CoA dehydrogenase deficiency in a 16-year-old girl with severe muscle wasting and scoliosis

1 reference

12 December 2020

The existence of two mitochondrial isoforms of 2,4-dienoyl-CoA reductase in the rat.

1 reference

12 December 2020

Hepatic and Muscular Presentations of Carnitine Palmitoyl Transferase Deficiency: Two Distinct Entities

1 reference

12 December 2020

A novel disease with deficiency of mitochondrial very-long-chain acyl-CoA dehydrogenase

1 reference

12 December 2020

Ketonuria and medium-chain acyl-CoA dehydrogenase deficiency

1 reference

12 December 2020

Purification and characterization of an alpha-methylacyl-CoA racemase from human liver

1 reference

12 December 2020

Mitochondrial very-long-chain acyl-coenzyme A dehydrogenase deficiency: clinical characteristics and diagnostic considerations in 30 patients

1 reference

12 December 2020

Combined enzyme defect of mitochondrial fatty acid oxidation

1 reference

12 December 2020

Analysis of carnitine esters by radio-high performance liquid chromatography in cultured skin fibroblasts from patients with mitochondrial fatty acid oxidation disorders

1 reference

12 December 2020

Molecular characterization of medium-chain acyl-CoA dehydrogenase (MCAD) deficiency: identification of a lys329 to glu mutation in the MCAD gene, and expression of inactive mutant enzyme protein in E. coli

1 reference

12 December 2020

Two alpha subunit donor splice site mutations cause human trifunctional protein deficiency

1 reference

12 December 2020

Cloning of glutaryl-CoA dehydrogenase cDNA, and expression of wild type and mutant enzymes in Escherichia coli

1 reference

12 December 2020

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.

1 reference

12 December 2020

Transient organic aciduria and persistent lacticacidemia in a patient with short-chain acyl-coenzyme A dehydrogenase deficiency

1 reference

12 December 2020

NADPH-dependent beta-oxidation of unsaturated fatty acids with double bonds extending from odd-numbered carbon atoms

1 reference

12 December 2020

A simple spectrophotometric assay for long-chain acyl-CoA dehydrogenase activity measurements in human skin fibroblasts

1 reference

12 December 2020

A new case of short-chain acyl-CoA dehydrogenase deficiency with isolated ethylmalonic aciduria

1 reference

12 December 2020

Clear correlation of genotype with disease phenotype in very-long-chain acyl-CoA dehydrogenase deficiency.

1 reference

12 December 2020

The existence of an inner-membrane-bound, long acyl-chain-specific 3-hydroxyacyl-CoA dehydrogenase in mammalian mitochondria

1 reference

12 December 2020

Normalization of short-chain acylcoenzyme A dehydrogenase after riboflavin treatment in a girl with multiple acylcoenzyme A dehydrogenase-deficient myopathy

1 reference

12 December 2020

Medium-chain acyl-CoA dehydrogenase deficiency. Diagnosis by stable-isotope dilution measurement of urinary n-hexanoylglycine and 3-phenylpropionylglycine

1 reference

12 December 2020

Mitochondropathy presenting with non-ketotic hypoglycaemia as 3-hydroxydicarboxylic aciduria

1 reference

12 December 2020

Short-chain acyl-CoA dehydrogenase deficiency associated with a lipid-storage myopathy and secondary carnitine deficiency.

1 reference

12 December 2020

Cloning of a cDNA for short/branched chain acyl-Coenzyme A dehydrogenase from rat and characterization of its tissue expression and substrate specificity

1 reference

12 December 2020

Molecular cloning of cDNA species for rat and mouse liver alpha-methylacyl-CoA racemases

1 reference

12 December 2020

Nucleotide sequence of medium-chain acyl-CoA dehydrogenase mRNA and its expression in enzyme-deficient human tissue

1 reference

12 December 2020

Quantitation of acyl-CoA and acylcarnitine esters accumulated during abnormal mitochondrial fatty acid oxidation

1 reference

12 December 2020

Mild or absent clinical signs in twin sisters with short-chain acyl-CoA dehydrogenase deficiency

1 reference

12 December 2020

Differential diagnosis of hydroxydicarboxylic aciduria based on release of3H2O from [9,10‐3H]myristic and [9,10‐3H]palmitic acids by intact cultured fibroblasts

1 reference

12 December 2020

Very long-chain acyl coenzyme A dehydrogenase deficiency presenting with exercise-induced myoglobinuria.

1 reference

12 December 2020

Short-chainL-3-hydroxyacyl-CoA dehydrogenase deficiency in muscle: A new cause for recurrent myoglobinuria and encephalopathy

1 reference

12 December 2020

Electron-transfer flavoprotein-ubiquinone oxidoreductase from pig liver: purification and molecular, redox, and catalytic properties

1 reference

12 December 2020

The occurrence of diastereomers of phytanic and pristanic acids and their determination by gas-liquid chromatography

1 reference

12 December 2020

A simple, straightforward assay for long-chain 3-hydroxyacyl-CoA dehydrogenase based on the use of N-ethylmaleimide: potential for pre- and postnatal diagnosis

1 reference

12 December 2020

Purification and properties of pig heart crotonase and the presence of short chain and long chain enoyl coenzyme A hydratases in pig and guinea pig tissues

1 reference

12 December 2020

Occurrence of a long-chain delta 3,delta 2-enoyl-CoA isomerase in rat liver

1 reference

12 December 2020

A new, simple assay for long-chain acyl-CoA dehydrogenase in cultured skin fibroblasts using stable isotopes and GC-MS

1 reference

12 December 2020

A spectrophotometric procedure for rapid and sensitive measurements of beta-oxidation. Demonstration of factors that can be rate-limiting for beta-oxidation

1 reference

12 December 2020

Isolation and characterization of cDNA for human 120 kDa mitochondrial 2,4-dienoyl-coenzyme A reductase

1 reference

12 December 2020

Isolation and characterization of rat and human cDNAs encoding a novel putative peroxisomal enoyl-CoA hydratase

1 reference

12 December 2020

Rat very-long-chain acyl-CoA dehydrogenase, a novel mitochondrial acyl-CoA dehydrogenase gene product, is a rate-limiting enzyme in long-chain fatty acid beta-oxidation system. cDNA and deduced amino acid sequence and distinct specificities of [...]

1 reference

12 December 2020

Separation and properties of five distinct acyl-CoA dehydrogenases from rat liver mitochondria. Identification of a new 2-methyl branched chain acyl-CoA dehydrogenase

1 reference

12 December 2020

Divergent sequences in the 5' region of cDNA suggest alternative splicing as a mechanism for the generation of carnitine acetyltransferases with different subcellular localizations

1 reference

12 December 2020

Purification and Properties of Rat Liver Acyl-CoA Dehydrogenases and Electron Transfer Flavoprotein1

1 reference

12 December 2020

Molecular cloning of cDNAs encoding rat and human medium-chain acyl-CoA dehydrogenase and assignment of the gene to human chromosome 1

1 reference

12 December 2020

On the mechanism of dehydrogenation of fatty acyl derivatives of coenzyme A. II. The electron-transferring flavoprotein

1 reference

12 December 2020

Purification of human very-long-chain acyl-coenzyme A dehydrogenase and characterization of its deficiency in seven patients

1 reference

12 December 2020

Peroxisomes contain delta 3,5,delta 2,4-dienoyl-CoA isomerase and thus possess all enzymes required for the beta-oxidation of unsaturated fatty acids by a novel reductase-dependent pathway

1 reference

12 December 2020

Molecular basis of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: identification of the major disease-causing mutation in the alpha-subunit of the mitochondrial trifunctional protein

2 references

12 December 2020

https://api.crossref.org/works/10.1023%2FA%3A1005504223140

7 January 2021

Trifunctional enzyme deficiency: adult presentation of a usually fatal beta-oxidation defect

1 reference

12 December 2020

Short-chain acyl-coenzyme A dehydrogenase deficiency. Clinical and biochemical studies in two patients

1 reference

12 December 2020

L-3-hydroxyacyl coenzyme A dehydrogenase from pig heart muscle. I. Purification and properties

1 reference

12 December 2020

Measurement of acyl-CoA dehydrogenase activity in cultured skin fibroblasts and blood platelets

1 reference

12 December 2020

Human liver long-chain 3-hydroxyacyl-coenzyme A dehydrogenase is a multifunctional membrane-bound beta-oxidation enzyme of mitochondria

1 reference

12 December 2020

Purification by affinity chromatography of 2,4-dienoyl-CoA reductases from bovine liver and Escherichia coli

1 reference

12 December 2020

Fatty acid oxidation in soluble systems of mammalian origin: the beginnings

1 reference

12 December 2020

Catalytic defect of medium-chain acyl-coenzyme A dehydrogenase deficiency. Lack of both cofactor responsiveness and biochemical heterogeneity in eight patients

1 reference

12 December 2020

2,6-Dimethylheptanoyl-CoA is a specific substrate for long-chain acyl-CoA dehydrogenase (LCAD): evidence for a major role of LCAD in branched-chain fatty acid oxidation

1 reference

12 December 2020

Two mitochondrial 3-hydroxyacyl-CoA dehydrogenases in bovine liver

1 reference

12 December 2020

Molecular cloning and nucleotide sequence of complementary DNAs encoding human short chain acyl-coenzyme A dehydrogenase and the study of the molecular basis of human short chain acyl-coenzyme A dehydrogenase deficiency

1 reference

12 December 2020

Immunochemical characterization of variant medium-chain acyl-CoA dehydrogenase in fibroblasts from patients with medium-chain acyl-CoA dehydrogenase deficiency

1 reference

12 December 2020

Long Chain Enoyl Coenzyme A Hydratase from Pig Heart

1 reference

12 December 2020

Purification and mechanism of delta 3,delta 5-t-2,t-4-dienoyl-CoA isomerase from rat liver.

1 reference

12 December 2020

Genomic and mutational analysis of the mitochondrial trifunctional protein beta-subunit (HADHB) gene in patients with trifunctional protein deficiency

1 reference

12 December 2020

In vitro fibroblast studies in a patient with C6-C10-dicarboxylic aciduria: evidence for a defect in general acyl-CoA dehydrogenase

1 reference

12 December 2020

Fluorometric assay of acyl-CoA dehydrogenases in normal and mutant human fibroblasts

1 reference

12 December 2020

Stereochemistry of peroxisomal and mitochondrial beta-oxidation of alpha-methylacyl-CoAs

1 reference

12 December 2020

cDNA cloning of mitochondrial delta 3, delta 2-enoyl-CoA isomerase of rat liver

1 reference

12 December 2020

Novel fatty acid beta-oxidation enzymes in rat liver mitochondria. I. Purification and properties of very-long-chain acyl-coenzyme A dehydrogenase

1 reference

12 December 2020

Identification and quantification of intermediates of unsaturated fatty acid metabolism in plasma of patients with fatty acid oxidation disorders.

1 reference

12 December 2020

Purification and properties of mitochondrial and peroxisomal 3-hydroxyacyl-CoA dehydrogenase from rat liver

1 reference

12 December 2020

Very long chain acyl-CoA dehydrogenase deficiency: successful treatment of acute cardiomyopathy

1 reference

12 December 2020

Tandem mass spectrometry: a new method for acylcarnitine profiling with potential for neonatal screening for inborn errors of metabolism

1 reference

12 December 2020

An acyl-coenzyme A dehydrogenase assay utilizing the ferricenium ion

1 reference

12 December 2020

Human short-chain L-3-hydroxyacyl-CoA dehydrogenase: cloning and characterization of the coding sequence

1 reference

12 December 2020

The fasting test in paediatrics: application to the diagnosis of pathological hypo- and hyperketotic states.

1 reference

12 December 2020

Properties of mitochondria and peroxisomal enoyl-CoA hydratases from rat liver

1 reference

12 December 2020

A comparison of [9,10-3H]palmitic and [9,10-3H]myristic acids for the detection of defects of fatty acid oxidation in intact cultured fibroblasts

1 reference

12 December 2020

Molecular cloning and sequence analysis of the cDNA for rat mitochondrial enoyl-CoA hydratase. Structural and evolutionary relationships linked to the bifunctional enzyme of the peroxisomal beta-oxidation system

1 reference

12 December 2020

Hypoketonuric 3-hydroxydicarboxylic aciduria in five patients with glycogen storage disease

1 reference

12 December 2020

Genetic deficiency of short-chain acyl-coenzyme A dehydrogenase in cultured fibroblasts from a patient with muscle carnitine deficiency and severe skeletal muscle weakness

1 reference

12 December 2020

Evidence for intermediate channeling in mitochondrial beta-oxidation

1 reference

12 December 2020

Assay of L-3-hydroxyacyl-coenzyme A dehydrogenase with substrates of different chain lengths

1 reference

12 December 2020

Short-chain acyl-CoA dehydrogenase deficiency: a cause of ophthalmoplegia and multicore myopathy

1 reference

12 December 2020

Mitochondrial very long-chain acyl-CoA dehydrogenase deficiency with a mild clinical course

1 reference

12 December 2020

NADPH-dependent reductive metabolism of cis-5 unsaturated fatty acids. A revised pathway for the beta-oxidation of oleic acid

1 reference

12 December 2020

The molecular basis of pediatric long chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with maternal acute fatty liver of pregnancy

1 reference

12 December 2020

Novel fatty acid beta-oxidation enzymes in rat liver mitochondria. II. Purification and properties of enoyl-coenzyme A (CoA) hydratase/3-hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase trifunctional protein

1 reference

12 December 2020

Medium chain 3-ketoacyl-coenzyme A thiolase deficiency: a new disorder of mitochondrial fatty acid beta-oxidation

1 reference

12 December 2020

Ethylmalonic aciduria is associated with an amino acid variant of short chain acyl-coenzyme A dehydrogenase

1 reference

12 December 2020

Purification and characterization of the trifunctional beta-oxidation complex from pig heart mitochondria

1 reference

12 December 2020

The long-chain acyl-CoA dehydrogenase deficiency

1 reference

12 December 2020

Purification and properties of an alpha-methylacyl-CoA racemase from rat liver

1 reference

12 December 2020

Diagnosis of inborn errors of metabolism from blood spots by acylcarnitines and amino acids profiling using automated electrospray tandem mass spectrometry

1 reference

12 December 2020

Quantitative plasma acylcarnitine analysis using electrospray tandem mass spectrometry for the diagnosis of organic acidaemias and fatty acid oxidation defects

1 reference

12 December 2020

The genes for the alpha and beta subunits of the mitochondrial trifunctional protein are both located in the same region of human chromosome 2p23

1 reference

12 December 2020

Molecular characterization of inherited medium-chain acyl-CoA dehydrogenase deficiency

1 reference

12 December 2020

Long-chain acyl coenzyme A dehydrogenase deficiency: an inherited cause of nonketotic hypoglycemia.

1 reference

12 December 2020

Purification and properties of a pig heart thiolase with broad chain length specificity and comparison of thiolases from pig heart and Escherichia coli

1 reference

12 December 2020

Structural organization of the human short-chain L-3-hydroxyacyl-CoA dehydrogenase gene

1 reference

12 December 2020

Cloning of human very-long-chain acyl-coenzyme A dehydrogenase and molecular characterization of its deficiency in two patients

1 reference

12 December 2020

The metabolism of phytanic acid and pristanic acid in man: a review

2 references

12 December 2020

https://api.crossref.org/works/10.1023%2FA%3A1005504223140

7 January 2021

Identification of two variant short chain acyl-coenzyme A dehydrogenase alleles, each containing a different point mutation in a patient with short chain acyl-coenzyme A dehydrogenase deficiency

1 reference

12 December 2020

Medium-chain acyl-coenzyme A dehydrogenase deficiency: clinical course in 120 affected children.

1 reference

12 December 2020

Molecular cloning and sequence of the complementary DNA encoding human mitochondrial acetoacetyl-coenzyme A thiolase and study of the variant enzymes in cultured fibroblasts from patients with 3-ketothiolase deficiency

1 reference

12 December 2020

Isolation and expression of a cDNA encoding the precursor for a novel member (ACADSB) of the acyl-CoA dehydrogenase gene family

1 reference

12 December 2020

Measurement of short-chain acyl-CoA dehydrogenase (SCAD) in cultured skin fibroblasts with hexanoyl-CoA as a competitive inhibitor to eliminate the contribution of medium-chain acyl-CoA dehydrogenase

1 reference

12 December 2020

Molecular cloning and nucleotide sequence of cDNA encoding the entire precursor of rat mitochondrial acetoacetyl-CoA thiolase

1 reference

12 December 2020

Immunochemical characterization of variant long-chain acyl-CoA dehydrogenase in cultured fibroblasts from nine patients with long-chain acyl-CoA dehydrogenase deficiency

1 reference

12 December 2020

Identification of four new mutations in the short-chain acyl-CoA dehydrogenase (SCAD) gene in two patients: one of the variant alleles, 511C-->T, is present at an unexpectedly high frequency in the general population, as was the case for 625G-->A, to

1 reference

12 December 2020

cDNA cloning of rat liver 2,4-dienoyl-CoA reductase

1 reference

12 December 2020

Quantitative acylcarnitine profiling in fibroblasts using [U-13C] palmitic acid: an improved tool for the diagnosis of fatty acid oxidation defects

1 reference

12 December 2020

Amino acid sequence of L-3-hydroxyacyl CoA dehydrogenase from pig heart muscle

1 reference

12 December 2020

Intramitochondrial localization of palmityl-CoA dehydrogenase, beta-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase in guinea-pig heart

1 reference

12 December 2020

A new iron-sulfur flavoprotein of the respiratory chain. A component of the fatty acid beta oxidation pathway

1 reference

12 December 2020

Structure and expression of the human mitochondrial acetoacetyl-CoA thiolase-encoding gene

1 reference

12 December 2020

Short-chain 3-hydroxy-2-methylacyl-CoA dehydrogenase from rat liver: purification and characterization of a novel enzyme of isoleucine metabolism

1 reference

12 December 2020

Deficiency of long-chain 3-hydroxyacyl-CoA dehydrogenase: a cause of lethal myopathy and cardiomyopathy in early childhood

1 reference

12 December 2020

Mitochondrial 3-2 trans -Enoyl-CoA Isomerase. Purification, Cloning, Expression, and Mitochondrial Import of the Key Enzyme of Unsaturated Fatty acid β -Oxidation

1 reference

12 December 2020

Enhancement of long-chain acyl-CoA hydrolase activity in peroxisomes and mitochondria of rat liver by peroxisomal proliferators

1 reference

12 December 2020

Molecular basis of medium chain acyl-coenzyme A dehydrogenase deficiency. An A to G transition at position 985 that causes a lysine-304 to glutamate substitution in the mature protein is the single prevalent mutation

1 reference

12 December 2020

Mitochondrial short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase deficiency: a new defect of fatty acid oxidation.

1 reference

12 December 2020

Crystallographic studies of 3-ketoacylCoA thiolase from yeast Saccharomyces cerevisiae

1 reference

12 December 2020

Phytanic acid and pristanic acid are oxidized by sequential peroxisomal and mitochondrial reactions in cultured fibroblasts

1 reference

12 December 2020

Screening blood spots for inborn errors of metabolism by electrospray tandem mass spectrometry with a microplate batch process and a computer algorithm for automated flagging of abnormal profiles.

1 reference

12 December 2020

Muscle Carnitine Palmityltransferase Deficiency and Myoglobinuria

1 reference

12 December 2020

Rapid degradation of short-chain acyl-CoA dehydrogenase variants with temperature-sensitive folding defects occurs after import into mitochondria

1 reference

12 December 2020

Purification and characterization of short-chain, medium-chain, and long-chain acyl-CoA dehydrogenases from rat liver mitochondria. Isolation of the holo- and apoenzymes and conversion of the apoenzyme to the holoenzyme

1 reference

12 December 2020

Measurement of the acyl-CoA intermediates of beta-oxidation by h.p.l.c. with on-line radiochemical and photodiode-array detection. Application to the study of [U-14C]hexadecanoate oxidation by intact rat liver mitochondria

1 reference

12 December 2020

Purification and characterization of bovine liver 3-cis-2-trans-enoyl-CoA isomerase

1 reference

12 December 2020

Inborn errors of metabolism diagnosed in sudden death cases by acylcarnitine analysis of postmortem bile

1 reference

12 December 2020

Very long chain acyl-coenzyme A dehydrogenase deficiency with adult onset

1 reference

12 December 2020

Infantile form of carnitine palmitoyltransferase II deficiency with hepatomuscular symptoms and sudden death. Physiopathological approach to carnitine palmitoyltransferase II deficiencies

1 reference

12 December 2020

Effects of two mutations detected in medium chain acyl-CoA dehydrogenase (MCAD)-deficient patients on folding, oligomer assembly, and stability of MCAD enzyme

1 reference

12 December 2020

Improved detection of long-chain fatty acid oxidation defects in intact cells using [9,10-3H]oleic acid

1 reference

12 December 2020

Mitochondrial beta-oxidation of 2-methyl fatty acids in rat liver

1 reference

12 December 2020

Purification and characterization of isovaleryl coenzyme A dehydrogenase from rat liver mitochondria

1 reference

12 December 2020

Degradation of unsaturated fatty acids. Identification of intermediates in the degradation of cis-4-decenoly-CoA by extracts of beef-liver mitochondria

1 reference

12 December 2020

cDNA-derived amino acid sequence of rat mitochondrial 3-oxoacyl-CoA thiolase with no transient presequence: structural relationship with peroxisomal isozyme

1 reference

12 December 2020

Molecular cloning and nucleotide sequence of cDNAs encoding human long-chain acyl-CoA dehydrogenase and assignment of the location of its gene (ACADL) to chromosome 2.

1 reference

12 December 2020

Mild trifunctional protein deficiency is associated with progressive neuropathy and myopathy and suggests a novel genotype-phenotype correlation

1 reference

12 December 2020

Purification and characterization of 2-methyl-branched chain acyl coenzyme A dehydrogenase, an enzyme involved in the isoleucine and valine metabolism, from rat liver mitochondria

1 reference

12 December 2020

Molecular cloning of cDNA for rat mitochondrial 3-oxoacyl-CoA thiolase

1 reference

12 December 2020

Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency with the G1528C mutation: clinical presentation of thirteen patients.

1 reference

12 December 2020

Hypoparathyroidism in mitochondrial trifunctional protein deficiency

1 reference

12 December 2020

Mutation analysis of very-long-chain acyl-coenzyme A dehydrogenase (VLCAD) deficiency: identification and characterization of mutant VLCAD cDNAs from four patients.

1 reference

12 December 2020

Purification and properties of 3-cis-2-trans-enoyl-CoA isomerase (dodecenoyl-CoA delta-isomerase) from rat liver mitochondria

2 references

12 December 2020

https://api.crossref.org/works/10.1023%2FA%3A1005504223140

7 January 2021

Diagnosis of medium-chain acyl-CoA dehydrogenase deficiency in lymphocytes and liver by a gas chromatographic method: the effect of oral riboflavin supplementation

1 reference

12 December 2020

The presence of a new 3-oxoacyl-CoA thiolase in rat liver peroxisomes

1 reference

12 December 2020

Molecular characterization of mitochondrial trifunctional protein deficiency: formation of the enzyme complex is important for stabilization of both alpha- and beta-subunits

1 reference

12 December 2020

Very long chain acyl-coenzyme A dehydrogenase deficiency in two siblings: evolution after prenatal diagnosis and prompt management

1 reference

12 December 2020

Delta 3,delta 2-enoyl-CoA isomerases. Characterization of the mitochondrial isoenzyme in the rat.

1 reference

12 December 2020

Delta3,5-delta2,4-dienoyl-CoA isomerase from rat liver. Molecular characterization

1 reference

12 December 2020

Molecular basis of human mitochondrial very-long-chain acyl-CoA dehydrogenase deficiency causing cardiomyopathy and sudden death in childhood

1 reference

12 December 2020

Mitochondrial very long chain acyl-CoA dehydrogenase deficiency--a new disorder of fatty acid oxidation

1 reference

12 December 2020

Very long chain acyl-CoA dehydrogenase deficiency: identification of a new inborn error of mitochondrial fatty acid oxidation in fibroblasts

1 reference

12 December 2020

Molecular cloning of the cDNAs for the subunits of rat mitochondrial fatty acid beta-oxidation multienzyme complex. Structural and functional relationships to other mitochondrial and peroxisomal beta-oxidation enzymes

1 reference

12 December 2020

Delta 3,5, delta 2,4-dienoyl-CoA isomerase from rat liver mitochondria. Purification and characterization of a new enzyme involved in the beta-oxidation of unsaturated fatty acids

1 reference

12 December 2020

Molecular cloning and sequence analysis of the cDNA for human mitochondrial short-chain enoyl-CoA hydratase

1 reference

12 December 2020

Hypoglycemia, hepatic dysfunction, muscle weakness, cardiomyopathy, free carnitine deficiency and long-chain acylcarnitine excess responsive to medium chain triglyceride diet.

1 reference

12 December 2020

Molecular cloning and nucleotide sequence of cDNAs encoding the precursors of rat long chain acyl-coenzyme A, short chain acyl-coenzyme A, and isovaleryl-coenzyme A dehydrogenases. Sequence homology of four enzymes of the acyl-CoA dehydrogenase fami

1 reference

12 December 2020

Structural analysis of cDNAs for subunits of human mitochondrial fatty acid beta-oxidation trifunctional protein

1 reference

12 December 2020

Identification of very-long-chain acyl-CoA dehydrogenase deficiency in three patients previously diagnosed with long-chain acyl-CoA dehydrogenase deficiency

1 reference

12 December 2020

Evidence of two catalytically active carnitine medium/long chain acyltransferases in rat liver peroxisomes

1 reference

12 December 2020

Cloning and characterization of human very-long-chain acyl-CoA dehydrogenase cDNA, chromosomal assignment of the gene and identification in four patients of nine different mutations within the VLCAD gene.

1 reference

12 December 2020

Medium-chain acyl-CoA dehydrogenase deficiency in children with non-ketotic hypoglycemia and low carnitine levels

1 reference

12 December 2020

Amino acid sequence similarities of the mitochondrial short chain delta 3, delta 2-enoyl-CoA isomerase and peroxisomal multifunctional delta 3, delta 2-enoyl-CoA isomerase, 2-enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase enzyme in rat liver. T

1 reference

12 December 2020

Short-Chain Acyl-Coenzyme A Dehydrogenase Activity, Antigen, and Biosynthesis Are Absent in the BALB/cByJ Mouse

1 reference

12 December 2020

Rapid diagnosis of MCAD deficiency: quantitative analysis of octanoylcarnitine and other acylcarnitines in newborn blood spots by tandem mass spectrometry

1 reference

12 December 2020

Cloning and sequence analysis of a full length cDNA encoding human mitochondrial 3-oxoacyl-CoA thiolase

1 reference

12 December 2020

Dicarboxylic aciduria: deficient [1-14C]octanoate oxidation and medium-chain acyl-CoA dehydrogenase in fibroblasts

1 reference

12 December 2020

Human trifunctional protein deficiency: A new disorder of mitochondrial fatty acid β-oridation

1 reference

12 December 2020

Clinical and biochemical characterization of short-chain acyl-coenzyme A dehydrogenase deficiency

1 reference

12 December 2020

Δ3,5-Δ2,4-Dienoyl-CoA Isomerase from Rat Liver

1 reference

https://api.crossref.org/works/10.1023%2FA%3A1005504223140

7 January 2021