butyric acid has been researched along with Propionic Acidemia in 1 studies
Butyric Acid: A four carbon acid, CH3CH2CH2COOH, with an unpleasant odor that occurs in butter and animal fat as the glycerol ester.
butyrate : A short-chain fatty acid anion that is the conjugate base of butyric acid, obtained by deprotonation of the carboxy group.
butyric acid : A straight-chain saturated fatty acid that is butane in which one of the terminal methyl groups has been oxidised to a 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 |
---|---|---|
"Propionic Acidemia (PA) is a rare metabolic disorder caused by the defect in enzyme (propionyl-coenzyme A (CoA) carboxylase) leading to the abnormal accumulation of metabolites of branched-chain amino acid catabolism in blood and urine." | 4.12 | A Rare Case of Propionic Acidemia in a Six Months Female Child. ( Aamir, M; Bibi, A; Chaudhry, N; Haroon, ZH; Irum, S; Rehman, A, 2022) |
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 | 0 (0.00) | 24.3611 |
2020's | 1 (100.00) | 2.80 |
Authors | Studies |
---|---|
Irum, S | 1 |
Rehman, A | 1 |
Aamir, M | 1 |
Haroon, ZH | 1 |
Chaudhry, N | 1 |
Bibi, A | 1 |
1 other study available for butyric acid and Propionic Acidemia
Article | Year |
---|---|
A Rare Case of Propionic Acidemia in a Six Months Female Child.
Topics: Amino Acids, Branched-Chain; Butyric Acid; Citrates; Coenzyme A; Female; Glycine; Humans; Infant; Ke | 2022 |