malic acid has been researched along with Propionic Acidemia in 2 studies
malic acid : A 2-hydroxydicarboxylic acid that is succinic acid in which one of the hydrogens attached to a carbon is replaced by a hydroxy group.
2-hydroxydicarboxylic acid : Any dicarboxylic acid carrying a hydroxy group on the carbon atom at position alpha to the carboxy group.
Propionic Acidemia: Autosomal recessive metabolic disorder caused by mutations in PROPIONYL-COA CARBOXYLASE genes that result in dysfunction of branch chain amino acids and of the metabolism of certain fatty acids. Neonatal clinical onset is characterized by severe metabolic acidemia accompanied by hyperammonemia, HYPERGLYCEMIA, lethargy, vomiting, HYPOTONIA; and HEPATOMEGALY. Survivors of the neonatal onset propionic acidemia often show developmental retardation, and intolerance to dietary proteins. Late-onset form of the disease shows mild mental and/or developmental retardation, sometimes without metabolic acidemia.
| Excerpt | Relevance | Reference |
|---|---|---|
| "Accumulation of 2-methylcitric acid (2MCA) is observed in methylmalonic and propionic acidemias, which are clinically characterized by severe neurological symptoms." | 3.83 | 2-Methylcitric acid impairs glutamate metabolism and induces permeability transition in brain mitochondria. ( Amaral, AU; Castilho, RF; Cecatto, C; Wajner, M, 2016) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 0 (0.00) | 29.6817 |
| 2010's | 1 (50.00) | 24.3611 |
| 2020's | 1 (50.00) | 2.80 |
| Authors | Studies |
|---|---|
| Roginski, AC | 1 |
| Zemniaçak, ÂB | 1 |
| Marschner, RA | 1 |
| Wajner, SM | 1 |
| Ribeiro, RT | 1 |
| Wajner, M | 2 |
| Amaral, AU | 2 |
| Cecatto, C | 1 |
| Castilho, RF | 1 |
2 other studies available for malic acid and Propionic Acidemia
| Article | Year |
|---|---|
Disruption of mitochondrial functions involving mitochondrial permeability transition pore opening caused by maleic acid in rat kidney.
Topics: Adenosine Diphosphate; Adenosine Triphosphate; Animals; Calcium; Cyclosporine; Glutamic Acid; HEK293 | 2022 |
2-Methylcitric acid impairs glutamate metabolism and induces permeability transition in brain mitochondria.
Topics: Adenosine Diphosphate; Adenosine Triphosphate; Amino Acid Metabolism, Inborn Errors; Animals; Brain; | 2016 |