The Molecular Basis of Malonyl-CoA Decarboxylase Deficiency

PROTEIN MEASUREMENT WITH THE FOLIN PHENOL REAGENT

1951 • 319,299 citations

Basic local alignment search tool

1990 • 87,358 citations

Methods of Enzymatic Analysis

1975 • 20,446 citations

Primer-Directed Enzymatic Amplification of DNA with a Thermostable DNA Polymerase

1988 • 17,059 citations

Methods of Enzymatic Analysis

1974 • 6,512 citations

Fluorescamine: A Reagent for Assay of Amino Acids, Peptides, Proteins, and Primary Amines in the Picomole Range

1972 • 2,632 citations

AN ELECTRON-TRANSPORT SYSTEM ASSOCIATED WITH THE OUTER MEMBRANE OF LIVER MITOCHONDRIA

1967 • 2,311 citations

Computational Method to Predict Mitochondrially Imported Proteins and their Targeting Sequences

1996 • 1,654 citations

A gene map of the human genome.

1996 • 986 citations

A Gene Map of the Human Genome

1996 • 737 citations

[2] Citrate synthase from rat liver

1969 • 435 citations

Peroxisomal protein import is conserved between yeast, plants, insects and mammals.

1990 • 296 citations

Mutagenically separated PCR (MS-PCR): a highly specific one step procedure for easy mutation detection

1993 • 251 citations

Docosahexaenoic Acid Is the Preferred Dietary n-3 Fatty Acid for the Development of the Brain and Retina

1990 • 201 citations

Docosahexaenoic acid synthesis in human skin fibroblasts involves peroxisomal retroconversion of tetracosahexaenoic acid

1995 • 160 citations

Characterization of cardiac malonyl-CoA decarboxylase and its putative role in regulating fatty acid oxidation

1998 • 135 citations

The intracellular and intramitochondrial distribution of malonyl-CoA decarboxylase and propionyl-CoA carboxylase in rat liver

1969 • 83 citations

Distinct long chain and very long chain fatty acyl CoA synthetases in rat liver peroxisomes and microsomes

1988 • 77 citations

Malonyl coenzyme a decarboxylase deficiency

1983 • 74 citations

Increased cardiac fatty acid uptake with dobutamine infusion in swine is accompanied by a decrease in malonyl CoA levels

1996 • 67 citations

Lipid-lowerjng diets enriched with monounsaturated or polyunsaturated fatty acids but low in saturated fatty acids have similar effects on serum lipid concentrations in hyperlipidemic patients

1994 • 63 citations

The incidence of PAX6 mutation in patients with simple aniridia: an evaluation of mutation detection in 12 cases.

1997 • 61 citations

Increased cardiac fatty acid uptake with dobutamine infusion in swine is accompanied by a decrease in malonyl CoA levels

1996 • 50 citations

A new case of malonyl coenzyme A decarboxylase deficiency presenting with cardiomyopathy

1997 • 50 citations

Malonyl-CoA decarboxylase from the uropygial gland of waterfowl: Purification, properties, immunological comparison, and role in regulating the synthesis of multimethyl-branched fatty acids

1978 • 50 citations

Malonyl coenzyme A decarboxylase deficiency. Clinical and biochemical findings in a second child with a more severe enzyme defect

1986 • 47 citations

Malonic aciduria and cardiomyopathy

1993 • 47 citations

Deletion of the Conserved First 18 N-Terminal Amino Acid Residues in Rat Liver Carnitine Palmitoyltransferase I Abolishes Malonyl-CoA Sensitivity and Binding

1998 • 42 citations

Malonyl coenzyme A decarboxylase deficiency.

1993 • 42 citations

Molecular Cloning, Nucleotide Sequence, and Tissue Distribution of Malonyl-CoA Decarboxylase

1989 • 35 citations

A simple incubation flask for 14CO2 collection

1971 • 33 citations

Malonyl-CoA inhibition of peroxisomal carnitine octanoyltransferase

1992 • 30 citations

Cytoplasmic accumulation of a normally mitochondrial malonyl-CoA decarboxylase by the use of an alternate transcription start site

1992 • 30 citations

Association of malonyl‐CoA decarboxylase deficiency and heterozygote state for haemoglobin C disease

1994 • 29 citations

Malonyl-CoA decarboxylase from the mammary gland of lactating rat

1978 • 22 citations

Studies on the Intracellular Localization of Acetyl-CoA Carboxylase

1997 • 21 citations

Inhibition of erythromycin synthesis by disruption of malonyl-coenzyme A decarboxylase gene eryM in Saccharopolyspora erythraea

1994 • 20 citations

Acetyl-CoA Carboxylase Is Essential for Nutrient-Induced Insulin Secretion

1996 • 19 citations

Developmental pattern of the expression of malonyl-CoA decarboxylase gene and the production of unique lipids in the goose uropygial glands

1987 • 15 citations

Regulation of carbohydrate and fatty acid utilization by L-carnitine during cardiac development and hypoxia.

1998 • 11 citations

The cellular localization of malonyl-coenzyme A decarboxylase in rat brain

1994 • 6 citations

Is mild deficiency of mitochondrial malonyl CoA decarboxylase a risk factor for hyperlipidemia?

1988 • 1 citations