Page last updated: 2024-10-16

butyric acid and Propionic Acidemia

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.

Research Excerpts

ExcerptRelevanceReference
"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."4.12A Rare Case of Propionic Acidemia in a Six Months Female Child. ( Aamir, M; Bibi, A; Chaudhry, N; Haroon, ZH; Irum, S; Rehman, A, 2022)

Research

Studies (1)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's0 (0.00)29.6817
2010's0 (0.00)24.3611
2020's1 (100.00)2.80

Authors

AuthorsStudies
Irum, S1
Rehman, A1
Aamir, M1
Haroon, ZH1
Chaudhry, N1
Bibi, A1

Other Studies

1 other study available for butyric acid and Propionic Acidemia

ArticleYear
A Rare Case of Propionic Acidemia in a Six Months Female Child.
    Journal of the College of Physicians and Surgeons--Pakistan : JCPSP, 2022, Volume: 32, Issue:8

    Topics: Amino Acids, Branched-Chain; Butyric Acid; Citrates; Coenzyme A; Female; Glycine; Humans; Infant; Ke

2022