|1.||Ryan, Robert O: 1 article (08/2015)|
|2.||Reis, Maria A M: 1 article (06/2014)|
|3.||Oliveira, Rui: 1 article (06/2014)|
|4.||Pardelha, Filipa: 1 article (06/2014)|
|5.||Albuquerque, Maria G E: 1 article (06/2014)|
|6.||Dias, João M L: 1 article (06/2014)|
|7.||Jacobsen, Ilse D: 1 article (03/2014)|
|8.||Otzen, Christian: 1 article (03/2014)|
|9.||Bardl, Bettina: 1 article (03/2014)|
|10.||Nett, Markus: 1 article (03/2014)|
10/01/2000 - "Despite minimal improvement in the levels of circulating propionyl CoA metabolites, hyperammonemia was corrected, and no episode of metabolic decompensation was experienced after the transplantation was performed. "
12/01/1979 - "A decreased level of N-acetylglutamate in liver mitochondria that would follow inhibition of N-acetylglutamate synthetase by propionyl-CoA would be expected to lead to hyperammonemia. "
01/01/2008 - "Hyperammonemia in patients with methylmalonic aciduria (MMA) and propionic aciduria (PA) is caused by accumulation of propionyl-CoA which decreases the synthesis of N-acetyl-glutamate, the natural activator of carbamyl phosphate synthetase 1. A treatment approach with carglumic acid, the structural analogue of N-acetyl-glutamate, has been proposed to decrease high ammonia levels encountered in MMA and PA crises. "
|2.||Metabolic Diseases (Metabolic Disease)
05/01/2007 - "The glial metabolism of propionate suggests astrocytic vulnerability in propionic acidemia when intramitochondrial propionyl-CoA may accumulate. "
05/01/2007 - "In propionic acidemia, propionate acts as a metabolic toxin in liver cells by accumulating in mitochondria as propionyl-CoA and its derivative, methylcitrate, two tricarboxylic acid cycle inhibitors. "
03/01/1995 - "When beta-ketopentanoate was infused at a rate corresponding to 75% of the dog's caloric requirement, the steady-state total plasma concentration of the two substrates was only 1.3 mM. Because the substrates are precursors of propionyl-CoA, we assayed the urinary concentrations of markers of propionic acidemia. "
05/01/1983 - "We conclude that the residual propionyl CoA carboxylating activity found in isolated PCC deficiency represents another carboxylase that can utilize propionyl CoA as a substrate rather than a mutant form of PCC with markedly different immunochemical and physicochemical properties."
02/01/1982 - "Liver tissues and fibroblasts from patients with propionic acidemia assigned to the pcc BC genetic complementation group have previously been shown to contain normal or near-normal quantities of structurally altered propionyl CoA carboxylases (PCC). "
|1.||Coenzyme A (CoA)
|2.||Acetyl Coenzyme A (Acetyl-CoA)
|3.||Pyruvic Acid (Pyruvate)
|4.||tiglyl-coenzyme A (methylcrotonyl-CoA)
|5.||Fatty Acids (Saturated Fatty Acids)
|6.||N-acetylglutamate (N-acetylglutamic acid)
|7.||propionyl-coenzyme A (propionyl-CoA)
|8.||Amino-Acid N-Acetyltransferase (N-Acetylglutamate Synthase)
|9.||Methylmalonyl-CoA Decarboxylase (Propionyl CoA Carboxylase)
|10.||Succinic Acid (Succinate)
|1.||Transplantation (Transplant Recipients)