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methylmalonic acid and Hyperammonemia

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.

Research Excerpts

ExcerptRelevanceReference
"In propionic aciduria and methylmalonic aciduria, hyperammonemia as a symptom of metabolic decompensation is one of the major clinical problems."7.76N-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."7.74Carglumic 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."3.83A 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."3.80Disruption 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."3.76N-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."3.74Carglumic 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)

Research

Studies (11)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's5 (45.45)29.6817
2010's5 (45.45)24.3611
2020's1 (9.09)2.80

Authors

AuthorsStudies
Choi, J1
Kim, JH1
Viegas, CM1
Zanatta, Â1
Grings, M1
Hickmann, FH1
Monteiro, WO1
Soares, LE1
Sitta, Â1
Leipnitz, G1
de Oliveira, FH1
Wajner, M1
Royes, LF1
Gabbi, P1
Ribeiro, LR1
Della-Pace, ID1
Rodrigues, FS1
de Oliveira Ferreira, AP1
da Silveira Junior, ME1
da Silva, LR1
Grisólia, AB1
Braga, DV1
Dobrachinski, F1
da Silva, AM1
Soares, FA1
Marchesan, S1
Furian, AF1
Oliveira, MS1
Fighera, MR1
Lee, NC1
Chien, YH1
Peng, SF1
Huang, AC1
Liu, TT1
Wu, AS1
Chen, LC1
Hsu, LW1
Tseng, SC1
Hwu, WL1
Filippi, L1
Gozzini, E1
Fiorini, P1
Malvagia, S1
la Marca, G1
Donati, MA1
von Stülpnagel, C1
Leichsenring, M1
Müller, A1
Staudt, M1
Kluger, G1
Watanabe, T1
Hoshi, K1
Zhang, C1
Ishida, Y1
Sakata, I1
Gebhardt, B1
Vlaho, S1
Fischer, D1
Sewell, A1
Böhles, H1
Lai, YC1
Huang, HP1
Tsai, IJ1
Tsau, YK1
Chen, CY1
Tsai, TC1
Lee, WJ1
Chen, HC1
Levrat, V1
Forest, I1
Fouilhoux, A1
Acquaviva, C1
Vianey-Saban, C1
Guffon, N1

Other Studies

11 other studies available for methylmalonic acid and Hyperammonemia

ArticleYear
Feline hyperammonemia associated with functional cobalamin deficiency: A case report.
    The Canadian veterinary journal = La revue veterinaire canadienne, 2023, Volume: 64, Issue:5

    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.
    Free radical research, 2014, Volume: 48, Issue:6

    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.
    Amino acids, 2016, Volume: 48, Issue:6

    Topics: Ammonia; Animals; Cerebral Cortex; Cytokines; Electroencephalography; Glutamic Acid; Glycine; Homeos

2016
Brain damage by mild metabolic derangements in methylmalonic acidemia.
    Pediatric neurology, 2008, Volume: 39, Issue:5

    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.
    Neonatology, 2010, Volume: 97, Issue:3

    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.
    Neuropediatrics, 2011, Volume: 42, Issue:2

    Topics: Anticonvulsants; Child, Preschool; Epilepsies, Partial; Humans; Hyperammonemia; Magnetic Resonance I

2011
Hyperammonaemia due to cobalamin malabsorption in a cat with exocrine pancreatic insufficiency.
    Journal of feline medicine and surgery, 2012, Volume: 14, Issue:12

    Topics: Animals; Cat Diseases; Cats; Exocrine Pancreatic Insufficiency; Hyperammonemia; Malabsorption Syndro

2012
N-carbamylglutamate enhances ammonia detoxification in a patient with decompensated methylmalonic aciduria.
    Molecular genetics and metabolism, 2003, Volume: 79, Issue:4

    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.
    Blood purification, 2007, Volume: 25, Issue:4

    Topics: Carbamoyl-Phosphate Synthase I Deficiency Disease; Child; Child, Preschool; Female; Hemofiltration;

2007
Continuous hemodiafiltration in the treatment of hyperammonemia due to methylmalonic acidemia.
    Renal failure, 2007, Volume: 29, Issue:6

    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?
    Orphanet journal of rare diseases, 2008, Jan-30, Volume: 3

    Topics: Amino Acid Metabolism, Inborn Errors; Female; Glutamates; Humans; Hyperammonemia; Infant, Newborn; M

2008