Methylmalonyl-CoA Decarboxylase (Propionyl CoA Carboxylase)

A carboxy-lyase that catalyzes the decarboxylation of (S)-2-Methyl-3-oxopropanoyl-CoA to propanoyl-CoA. In microorganisms the reaction can be coupled to the vectorial transport of SODIUM ions across the cytoplasmic membrane.

Also Known As:

Networked: 89 relevant articles (0 outcomes, 1 trials/studies)

Bio-Agent Context: Research Results

Experts

1.	Ugarte, M: 7 articles (01/2012 - 12/2001)
2.	Desviat, Lourdes R: 6 articles (09/2014 - 11/2002)
3.	Pérez, Belén: 5 articles (09/2014 - 11/2002)
4.	Ugarte, Magdalena: 5 articles (09/2014 - 11/2002)
5.	Pérez-Cerdá, Celia: 5 articles (01/2014 - 11/2002)
6.	Desviat, L R: 5 articles (09/2004 - 12/2001)
7.	Rodríguez-Pombo, P: 5 articles (09/2004 - 12/2001)
8.	Pérez, B: 5 articles (09/2004 - 12/2001)
9.	Sass, Jörn Oliver: 4 articles (07/2014 - 02/2007)
10.	Pérez-Cerdá, C: 4 articles (09/2004 - 12/2001)

Related Diseases

1.	Propionic Acidemia 02/28/1980 - "Immunological studies of propionyl CoA carboxylase in livers and fibroblasts of patients with propionic acidemia." 09/01/2014 - "Propionic acidemia (PA) is an autosomal recessive inborn error of metabolism caused by deficiency of propionyl-CoA carboxylase (PCC). " 07/01/2014 - "Propionic acidemia (PA) is a rare autosomal recessive organic aciduria resulting from defects in propionyl-CoA-carboxylase (PCC), a key enzyme of intermediate energy metabolism. " 02/01/2014 - "Propionic acidemia (PA) is caused by a deficiency of propionyl CoA carboxylase. " 09/01/2013 - "Propionic acidemia (PA) is a rare autosomal recessive disorder of metabolism caused by deficient activity of the mitochondrial enzyme propionyl-CoA carboxylase. "
2.	Metabolic Diseases (Metabolic Disease) 01/01/2014 - "Propionic acidemia is a metabolic disease produced by a deficiency of the enzyme propionyl-CoA carboxylase. " 10/01/1993 - "Propionic acidemia is an autosomal recessive metabolic disease resulting from a deficiency of propionyl CoA carboxylase (PCC) activity. " 05/01/1991 - "Propionic acidemia is an autosomal recessive metabolic disease resulting from a deficiency of propionyl CoA carboxylase (PCC) activity. " 12/01/1987 - "Propionicacidemia is an autosomal recessive metabolic disease resulting from a deficiency of propionyl-CoA carboxylase (PCC) activity. " 03/30/1999 - "The inherited metabolic disease propionic acidemia (PA) can result from mutations in either of the genes PCCA or PCCB, which encode the alpha and beta subunits, respectively, of the mitochondrial enzyme propionyl CoA-carboxylase. "
3.	Multiple Carboxylase Deficiency (Deficiency, Multiple Carboxylase) 01/01/1988 - "1. The multiple carboxylase deficiencies are inborn errors in the metabolism of biotin in which there is defective activity of propionyl CoA carboxylase, 3-methylcrotonyl CoA carboxylase and pyruvate carboxylase. " 01/01/1984 - "In biotin-responsive multiple carboxylase deficiency, a characteristic organic aciduria reflects in vivo deficiency of mitochondrial propionyl CoA carboxylase, 3-methylcrotonyl CoA carboxylase, and pyruvate carboxylase. " 09/01/1981 - "Three biotin-dependent enzymes, pyruvate carboxylase (PC), propionyl CoA carboxylase (PCC), and beta-methylcrotonyl CoA carboxylase (beta MCC), were biochemically characterized in fibroblasts from two patients with neonatal multiple carboxylase deficiency. " 07/01/1981 - "Multiple carboxylase deficiency is characterized by deficient activities of three biotin-dependent enzymes, propionyl coenzyme A carboxylase, pyruvate carboxylase, and beta-methylcrotonyl coenzyme A carboxylase. " 04/09/1981 - "Multiple carboxylase deficiency has previously been characterized by deficient activity of three biotin-dependent enzymes: propionyl CoA carboxylase, pyruvate carboxylase and beta-methylcrotonyl CoA carboxylase. "
4.	Hyperammonemia 08/01/2002 - "Propionic acidemia [MIM 606054] is a form of organic acidemia caused by genetic deficiency of propionyl-CoA carboxylase (PCC) and characterized by attacks of severe metabolic acidemia and hyperammonemia beginning in the neonatal period or in early infancy. " 01/01/2013 - "In this brief report, we describe the occurrence of autism in a 7-year-old girl with propionic acidemia (PA), a common form of organic aciduria resulting from the deficiency of propionyl-CoA carboxylase and characterized by frequent and potentially lethal episodes of metabolic acidosis often accompanied by hyperammonemia. "
5.	Inborn Errors Metabolism (Inborn Errors of Metabolism) 08/25/2002 - "Propionic acidemia is one of the most frequent inborn errors of metabolism caused by a deficiency of propionyl-CoA carboxylase. " 01/01/2014 - "Methylmalonic and propionic acidemia (MMA/PA) are inborn errors of metabolism characterized by accumulation of propionic acid and/or methylmalonic acid due to deficiency of methylmalonyl-CoA mutase (MUT) or propionyl-CoA carboxylase (PCC). "

Related Drugs and Biologics

1.	Biotin (Vitamin H)
2.	6-propylchromone-2-carboxylic acid (PCCA)
3.	Pyruvate Carboxylase
4.	Enzymes
5.	methylcrotonoyl-CoA carboxylase
6.	Methylmalonyl-CoA Mutase
7.	propionic acid (potassium propionate)
8.	Fatty Acids (Saturated Fatty Acids)
9.	Vitamins
10.	Proteins (Proteins, Gene)

Related Therapies and Procedures

1.	Liver Transplantation