Immunochemical Characterization of Variant Medium-Chain Acyl-CoA Dehydrogenase in Fibroblasts from Patients with Medium-Chain Acyl-CoA Dehydrogenase Deficiency

Cleavage of Structural Proteins during the Assembly of the Head of Bacteriophage T4

1970 • 253,849 citations

Measurement of protein using bicinchoninic acid

1985 • 17,516 citations

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.

1985 • 217 citations

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

1982 • 168 citations

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.

1990 • 99 citations

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.

1990 • 77 citations

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

1991 • 70 citations

Molecular heterogeneity of variant isovaleryl-CoA dehydrogenase from cultured isovaleric acidemia fibroblasts.

1985 • 35 citations

Short chain acyl-coenzyme A dehydrogenase (SCAD) deficiency. Immunochemical demonstration of molecular heterogeneity due to variant SCAD with differing stability.

1989 • 28 citations

Immunochemical Characterization of Variant Long-Chain Acyl-CoA Dehydrogenase in Cultured Fibroblasts from Nine Patients with Long-Chain Acyl-CoA Dehydrogenase Deficiency

1991 • 24 citations

Molecular analysis of medium chain acyl-CoA dehydrogenase deficiency.

1990 • 9 citations

Immunochemical and molecular analysis of medium-chain acyl CoA dehydrogenase deficiency.

1990 • 7 citations

Structure of the medium-chain acyl-CoA dehydrogenase from pig liver mitochondria at 3-Å resolution

1989 • 3 citations