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As a result, propionyl-CoA, propionic acid, ketones, ammonia, and other toxic compounds accumulate in the blood, causing the signs and symptoms of propionic acidemia. Hyperammonemia develops due to the inhibitory effects of propionyl-CoA on N-acetylglutamate synthase, indirectly resulting in slowing of the urea cycle. [12]
Typically, the parameter methylmalonic acid is only tested if propionylcarnitine was previously elevated. [48] Because of the inability to properly break down amino acids completely, the byproduct of protein digestion, the compound methylmalonic acid, is found in a disproportionate concentration in the blood and urine of those afflicted.
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 ...
This causes a buildup of propionic and/or methylmalonic acid, which has effects on infants ranging from severe brain damage to death. [2] The disease is linked to Vitamin B 12 , which is a cofactor for the enzyme methylmalonyl-CoA mutase.
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]
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 ...
Propionic acid: Propanoic acid CH 3 CH 2 COOH C3:0 Butyric acid: Butanoic acid CH 3 (CH 2) 2 COOH C4:0 Valeric acid: Pentanoic acid CH 3 (CH 2) 3 COOH C5:0 Caproic acid: Hexanoic acid CH 3 (CH 2) 4 COOH C6:0 Enanthic acid: Heptanoic acid CH 3 (CH 2) 5 COOH C7:0 Caprylic acid: Octanoic acid CH 3 (CH 2) 6 COOH C8:0 Pelargonic acid: Nonanoic acid ...
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]