Malonyl coenzyme a decarboxylase deficiency
- 28 April 1983
- journal article
- research article
- Published by Wiley in Journal of Inherited Metabolic Disease
- Vol. 7 (1) , 21-26
- https://doi.org/10.1007/bf01805615
Abstract
A patient is described with a deficiency of the mitochondrial enzyme, malonyl CoA decarboxylase — an inborn error of metabolism not recognized previously. The enzyme defect was first suspected because of persistent excretion of malonic and methylmalonic acids in urine in a child with repeated episodes of vomiting, some requiring hospitalization. Disturbances of lipid metabolism were demonstrated.This publication has 18 references indexed in Scilit:
- Malonyl-CoA decarboxylase from the mammary gland of lactating ratBiochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1978
- Purification and properties of malonyl-CoA decarboxylase from rat liver mitochondria and its immunological comparison with the enzymes from rat brain, heart, and mammary glandArchives of Biochemistry and Biophysics, 1978
- In situ detection of mycoplasma contamination in cell cultures by fluorescent Hoechst 33258 stainExperimental Cell Research, 1977
- Malonate metabolism in rat brain mitochondriaBiochemistry, 1974
- Rapid separation of particulate components and soluble cytoplasm of isolated rat-liver cellsBiochimica et Biophysica Acta (BBA) - Bioenergetics, 1974
- A direct radioassay of methylmalonyl-coenzyme A mutase using enzymatically synthesized dl-[3-14C]methylmalonyl-CoAAnalytical Biochemistry, 1973
- Properties of Malonyl‐CoA Decarboxylase and its Relation with Malonyl‐CoA Incorporation into Fatty Acids by Rat Liver MitochondriaEuropean Journal of Biochemistry, 1971
- The intracellular and intramitochondrial distribution of malonyl-CoA decarboxylase and propionyl-CoA carboxylase in rat liverBiochimica et Biophysica Acta (BBA) - Enzymology, 1969
- On the synthesis of malonyl-coenzyme A in rabbit-heart sarcosomesBiochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1966
- Untersuchungen uber die Harnstoffbildung im TierkörperHoppe-Seyler´s Zeitschrift Für Physiologische Chemie, 1932