Propionyl-Coenzyme A Carboxylase (Propionyl CoA Carboxylase)

Mitochondrial carboxylase that is involved in the catabolism of odd chain fatty acids and branched amino acids. Dysfunction of propionyl-CoA carboxylase leads to the inherited metabolic disorder PROPIONIC ACIDEMIA. In bacteria it is an ATP-dependent carboxylase in CO2 fixation reactions where propionyl-CoA is converted to (S)-methylmalonyl-CoA.

Also Known As:

Networked: 168 relevant articles (0 outcomes, 5 trials/studies)

Bio-Agent Context: Research Results

Experts

1.	Pérez, Belén: 9 articles (01/2021 - 11/2002)
2.	Desviat, Lourdes R: 9 articles (01/2020 - 11/2002)
3.	Pérez-Cerdá, Celia: 7 articles (01/2021 - 11/2002)
4.	Desviat, L R: 7 articles (01/2020 - 12/2001)
5.	Ugarte, Magdalena: 7 articles (01/2020 - 11/2002)
6.	Ugarte, M: 7 articles (01/2012 - 12/2001)
7.	Richard, Eva: 6 articles (01/2021 - 09/2013)
8.	Pérez, B: 6 articles (01/2016 - 12/2001)
9.	Mock, Donald M: 5 articles (01/2012 - 07/2002)
10.	Rodríguez-Pombo, P: 5 articles (09/2004 - 12/2001)

Related Diseases

1.	Biotin deficiency 02/01/1977 - "Biotin deficiency in the cat and the effect on hepatic propionyl CoA carboxylase." 08/01/2006 - "Lymphocyte propionyl-CoA carboxylase and its activation by biotin are sensitive indicators of marginal biotin deficiency in humans." 08/01/2002 - "Lymphocyte propionyl-CoA carboxylase and accumulation of odd-chain fatty acid in plasma and erythrocytes are useful indicators of marginal biotin deficiency small star, filled." 11/01/1977 - "Biotin deficiency progressively decreased both pyruvate and propionyl CoA carboxylase activities in liver, kidney, heart and brain. " 11/01/1977 - "Activities of pyruvate and propionyl CoA carboxylase in chicken tissues during normal growth and biotin deficiency were investigated. "
2.	Propionic Acidemia 02/28/1980 - "Immunological studies of propionyl CoA carboxylase in livers and fibroblasts of patients with propionic acidemia." 09/06/2022 - "Objective: Propionic acidemia is a rare inherited metabolic disorder caused by propionyl CoA carboxylase (PCC) deficiency. " 09/01/2022 - "Propionic acidemia (PA) is a metabolic disorder that involves a defective copy of propionyl-CoA carboxylase (PCC). " 01/01/2021 - "Propionic acidemia is a rare autosomal recessive inborn error of metabolism caused by a deficiency of propionyl CoA carboxylase which often manifests with frequent metabolic decompensations and risk of neurological injury. " 01/01/2021 - "Propionic acidemia (PA), an inborn error of metabolism, is caused by a deficiency in propionyl-CoA carboxylase. "
3.	Metabolic Diseases (Metabolic Disease) 04/01/2023 - "Propionic acidemia (PA) is an autosomal recessive inheritable metabolic disease caused by mutations in the propionyl CoA carboxylase gene (PCC) that affects multiple systems of the human body. " 01/01/2022 - "Propionic acidemia (PA) is a severe autosomal recessive metabolic disease caused by deficiency of propionyl-CoA carboxylase (PCC). " 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. "
4.	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. "
5.	Methylmalonic acidemia 12/01/2019 - "Methylmalonic acidemia (MMA) and propionic acidemia (PA) are related disorders of mitochondrial propionate metabolism, caused by defects in methylmalonyl-CoA mutase (MUT) and propionyl-CoA carboxylase (PCC), respectively. " 01/01/2019 - "Deficiency of propionyl-CoA carboxylase causes propionic acidemia and deficiencies of methylmalonyl-CoA mutase or its cofactor adenosylcobalamin cause methylmalonic acidemia. " 09/01/2006 - "elegans, including propionyl-CoA carboxylase subunits A and B (pcca-1, pccb-1), methylmalonic acidemia cobalamin A complementation group (mmaa-1), co(I)balamin adenosyltransferase (mmab-1), MMACHC (cblc-1), methylmalonyl-CoA epimerase (mce-1) and methylmalonyl-CoA mutase (mmcm-1) were identified. " 06/01/1977 - "Measurement of methylmalonyl-CoA mutase and propionyl-CoA carboxylase activities in lysates from fibroblasts derived from control, nonketotic hyperglycinemia, propionic acidemia, and both vitamin B12-responsive and -nonresponsive variants of methylmalonic acidemia showed only one abnormality: a 59% decrease in carboxylase activity in the nonketotic hyperglycinemic lysates (P less than 0.01). " 01/01/2020 - "Propionic acidemia (PA) and methylmalonic acidemia (MMA) are autosomal recessive disorders of propionyl-CoA (P-CoA) catabolism, which are caused by a deficiency in the enzyme propionyl-CoA carboxylase or the enzyme methylmalonyl-CoA (MM-CoA) mutase, respectively. "

Related Drugs and Biologics

1.	Biotin (Vitamin H)
2.	beta-hydroxyisovaleric acid (beta-hydroxy-beta-methylbutyrate)
3.	methylcrotonoyl-CoA carboxylase
4.	Enzymes
5.	Indicators and Reagents (Reagents)
6.	Carnitine (L-Carnitine)
7.	bisnorbiotin
8.	6-propylchromone-2-carboxylic acid (PCCA)
9.	methylmalonyl-coenzyme A (methylmalonyl CoA)
10.	propionic acid (potassium propionate)

Related Therapies and Procedures

1.	Protein-Restricted Diet (Diet, Protein Restricted)
2.	Liver Transplantation