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In many cases, propionic acidemia can damage the brain, heart, kidney, liver, cause seizures and delays to normal development such as walking or talking. The accumulation of propionic acid is known to induce differential responses in different organs. The heart and liver are specific targets of the complication.
Propionate fermentation is a form of fermentation with propionic acid as one of the products. This process is done through the fermentation pathway of bacteria. It is used in a variety of industrial, food-making, and medical applications.
One approach to reduce the accumulating amount of malonic acid and methylmalonic acid is diet. According to the state of knowledge in 1998, a high-carbohydrate and low-protein diet is recommended. [8] Changes in malonic acid and methylmalonic acid excretion can be seen as early as 24-36 h after a change in diet. [8]
Propionic acid inhibits the growth of mold and some bacteria at levels between 0.1 and 1% by weight. As a result, some propionic acid produced is consumed as a preservative for both animal feed and food for human consumption. For animal feed, it is used either directly or as its ammonium salt. This application accounts for about half of the ...
Acetic acid: Ethanoic acid Acetate: Ethanoate C 2 H 4 O 2: CH 3 COOH: 60.05 C3:0 Propionic acid: Propanoic acid Propionate: Propanoate C 3 H 6 O 2: CH 3 CH 2 COOH: 74.08 C4:0 Butyric acid: Butanoic acid Butyrate: Butanoate C 4 H 8 O 2: CH 3 (CH 2) 2 COOH: 88.11 C4:0 Isobutyric acid: 2-Methylpropanoic acid Isobutyrate: 2-Methylpropanoate C 4 H 8 ...
As with most other fatty acid oxidation disorders, individuals with MCADD need to avoid fasting for prolonged periods of time. During illnesses, they require careful management to stave off metabolic decompensation, which can result in death. [2] Supplementation of simple carbohydrates or glucose during illness is key to prevent catabolism. [3]
A deficiency is associated with propionic acidemia. [12] [13] [14] PCC activity is the most sensitive indicator of biotin status tested to date. In future pregnancy studies, the use of lymphocyte PCC activity data should prove valuable in assessment of biotin status. [15]
Propionyl-CoA is a coenzyme A derivative of propionic acid.It is composed of a 24 total carbon chain (without the coenzyme, it is a 3 carbon structure) and its production and metabolic fate depend on which organism it is present in. [1] Several different pathways can lead to its production, such as through the catabolism of specific amino acids or the oxidation of odd-chain fatty acids. [2]