methylmalonic acid has been researched along with Hyperammonemia in 11 studies
Methylmalonic Acid: A malonic acid derivative which is a vital intermediate in the metabolism of fat and protein. Abnormalities in methylmalonic acid metabolism lead to methylmalonic aciduria. This metabolic disease is attributed to a block in the enzymatic conversion of methylmalonyl CoA to succinyl CoA.
methylmalonic acid : A dicarboxylic acid that is malonic acid in which one of the methylene hydrogens is substituted by a methyl group.
Hyperammonemia: Elevated level of AMMONIA in the blood. It is a sign of defective CATABOLISM of AMINO ACIDS or ammonia to UREA.
Excerpt | Relevance | Reference |
---|---|---|
"In propionic aciduria and methylmalonic aciduria, hyperammonemia as a symptom of metabolic decompensation is one of the major clinical problems." | 7.76 | N-carbamylglutamate in emergency management of hyperammonemia in neonatal acute onset propionic and methylmalonic aciduria. ( Donati, MA; Filippi, L; Fiorini, P; Gozzini, E; la Marca, G; Malvagia, S, 2010) |
"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." | 7.74 | Carglumic acid: an additional therapy in the treatment of organic acidurias with hyperammonemia? ( Acquaviva, C; Forest, I; Fouilhoux, A; Guffon, N; Levrat, V; Vianey-Saban, C, 2008) |
"Hyperammonemia is a common finding in children with methylmalonic acidemia." | 3.83 | A neuronal disruption in redox homeostasis elicited by ammonia alters the glycine/glutamate (GABA) cycle and contributes to MMA-induced excitability. ( Braga, DV; da Silva, AM; da Silva, LR; da Silveira Junior, ME; de Oliveira Ferreira, AP; Della-Pace, ID; Dobrachinski, F; Fighera, MR; Furian, AF; Gabbi, P; Grisólia, AB; Marchesan, S; Oliveira, MS; Ribeiro, LR; Rodrigues, FS; Royes, LF; Soares, FA, 2016) |
"Hyperammonemia is a common finding in children with methylmalonic acidemia and propionic acidemia, but its contribution to the development of the neurological symptoms in the affected patients is poorly known." | 3.80 | Disruption of redox homeostasis and brain damage caused in vivo by methylmalonic acid and ammonia in cerebral cortex and striatum of developing rats. ( de Oliveira, FH; Grings, M; Hickmann, FH; Leipnitz, G; Monteiro, WO; Sitta, Â; Soares, LE; Viegas, CM; Wajner, M; Zanatta, Â, 2014) |
"In propionic aciduria and methylmalonic aciduria, hyperammonemia as a symptom of metabolic decompensation is one of the major clinical problems." | 3.76 | N-carbamylglutamate in emergency management of hyperammonemia in neonatal acute onset propionic and methylmalonic aciduria. ( Donati, MA; Filippi, L; Fiorini, P; Gozzini, E; la Marca, G; Malvagia, S, 2010) |
"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." | 3.74 | Carglumic acid: an additional therapy in the treatment of organic acidurias with hyperammonemia? ( Acquaviva, C; Forest, I; Fouilhoux, A; Guffon, N; Levrat, V; Vianey-Saban, C, 2008) |
Timeframe | Studies, this research(%) | All Research% |
---|---|---|
pre-1990 | 0 (0.00) | 18.7374 |
1990's | 0 (0.00) | 18.2507 |
2000's | 5 (45.45) | 29.6817 |
2010's | 5 (45.45) | 24.3611 |
2020's | 1 (9.09) | 2.80 |
Authors | Studies |
---|---|
Choi, J | 1 |
Kim, JH | 1 |
Viegas, CM | 1 |
Zanatta, Â | 1 |
Grings, M | 1 |
Hickmann, FH | 1 |
Monteiro, WO | 1 |
Soares, LE | 1 |
Sitta, Â | 1 |
Leipnitz, G | 1 |
de Oliveira, FH | 1 |
Wajner, M | 1 |
Royes, LF | 1 |
Gabbi, P | 1 |
Ribeiro, LR | 1 |
Della-Pace, ID | 1 |
Rodrigues, FS | 1 |
de Oliveira Ferreira, AP | 1 |
da Silveira Junior, ME | 1 |
da Silva, LR | 1 |
Grisólia, AB | 1 |
Braga, DV | 1 |
Dobrachinski, F | 1 |
da Silva, AM | 1 |
Soares, FA | 1 |
Marchesan, S | 1 |
Furian, AF | 1 |
Oliveira, MS | 1 |
Fighera, MR | 1 |
Lee, NC | 1 |
Chien, YH | 1 |
Peng, SF | 1 |
Huang, AC | 1 |
Liu, TT | 1 |
Wu, AS | 1 |
Chen, LC | 1 |
Hsu, LW | 1 |
Tseng, SC | 1 |
Hwu, WL | 1 |
Filippi, L | 1 |
Gozzini, E | 1 |
Fiorini, P | 1 |
Malvagia, S | 1 |
la Marca, G | 1 |
Donati, MA | 1 |
von Stülpnagel, C | 1 |
Leichsenring, M | 1 |
Müller, A | 1 |
Staudt, M | 1 |
Kluger, G | 1 |
Watanabe, T | 1 |
Hoshi, K | 1 |
Zhang, C | 1 |
Ishida, Y | 1 |
Sakata, I | 1 |
Gebhardt, B | 1 |
Vlaho, S | 1 |
Fischer, D | 1 |
Sewell, A | 1 |
Böhles, H | 1 |
Lai, YC | 1 |
Huang, HP | 1 |
Tsai, IJ | 1 |
Tsau, YK | 1 |
Chen, CY | 1 |
Tsai, TC | 1 |
Lee, WJ | 1 |
Chen, HC | 1 |
Levrat, V | 1 |
Forest, I | 1 |
Fouilhoux, A | 1 |
Acquaviva, C | 1 |
Vianey-Saban, C | 1 |
Guffon, N | 1 |
11 other studies available for methylmalonic acid and Hyperammonemia
Article | Year |
---|---|
Feline hyperammonemia associated with functional cobalamin deficiency: A case report.
Topics: Amino Acids; Ammonia; Animals; Cat Diseases; Cats; Female; Hyperammonemia; Methylmalonic Acid; Urea; | 2023 |
Disruption of redox homeostasis and brain damage caused in vivo by methylmalonic acid and ammonia in cerebral cortex and striatum of developing rats.
Topics: Ammonia; Animals; Antioxidants; Catalase; Cerebral Cortex; Corpus Striatum; Fluoresceins; Glutathion | 2014 |
A neuronal disruption in redox homeostasis elicited by ammonia alters the glycine/glutamate (GABA) cycle and contributes to MMA-induced excitability.
Topics: Ammonia; Animals; Cerebral Cortex; Cytokines; Electroencephalography; Glutamic Acid; Glycine; Homeos | 2016 |
Brain damage by mild metabolic derangements in methylmalonic acidemia.
Topics: Brain; Child, Preschool; Humans; Hyperammonemia; Infant; Infant, Newborn; Magnetic Resonance Imaging | 2008 |
N-carbamylglutamate in emergency management of hyperammonemia in neonatal acute onset propionic and methylmalonic aciduria.
Topics: Acute Disease; Amino Acid Metabolism, Inborn Errors; Ammonia; Emergency Medical Services; Female; Gl | 2010 |
Refractory focal epilepsy in a patient with methylmalonic aciduria: case report on positive and long-lasting effect of rufinamide.
Topics: Anticonvulsants; Child, Preschool; Epilepsies, Partial; Humans; Hyperammonemia; Magnetic Resonance I | 2011 |
Hyperammonaemia due to cobalamin malabsorption in a cat with exocrine pancreatic insufficiency.
Topics: Animals; Cat Diseases; Cats; Exocrine Pancreatic Insufficiency; Hyperammonemia; Malabsorption Syndro | 2012 |
N-carbamylglutamate enhances ammonia detoxification in a patient with decompensated methylmalonic aciduria.
Topics: Amino Acid Metabolism, Inborn Errors; Glutamates; Humans; Hyperammonemia; Inactivation, Metabolic; M | 2003 |
High-volume continuous venovenous hemofiltration as an effective therapy for acute management of inborn errors of metabolism in young children.
Topics: Carbamoyl-Phosphate Synthase I Deficiency Disease; Child; Child, Preschool; Female; Hemofiltration; | 2007 |
Continuous hemodiafiltration in the treatment of hyperammonemia due to methylmalonic acidemia.
Topics: Amino Acid Metabolism, Inborn Errors; Hemodiafiltration; Hemofiltration; Humans; Hyperammonemia; Inf | 2007 |
Carglumic acid: an additional therapy in the treatment of organic acidurias with hyperammonemia?
Topics: Amino Acid Metabolism, Inborn Errors; Female; Glutamates; Humans; Hyperammonemia; Infant, Newborn; M | 2008 |