Propionate citric acid cycle

In propionic acidemia , a rare inherited genetic disorder, propionate acts as a metabolic toxin in liver cells by accumulating in mitochondria as propionyl-CoA and its derivative, methylcitrate, two tricarboxylic acid cycle inhibitors. Propanoate is metabolized oxidatively by glia , which suggests astrocytic vulnerability in propionic acidemia when intramitochondrial propionyl-CoA may accumulate. Propionic acidemia may alter both neuronal and glial gene expression by affecting histone acetylation. [19] [20] When propionic acid is infused directly into rodents' brains, it produces reversible behavior (., hyperactivity , dystonia , social impairment, perseveration ) and brain changes (., innate neuroinflammation, glutathione depletion) that may be used as a means to model autism in rats. [19]

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TESTS
Solution S
Dissolve g in 80 ml of boiling distilled water, cool and dilute to 100 ml with  carbon dioxide-free water prepared from distilled water.
Appearance of solution
Solution S is clear and colourless.
pH
The pH of solution S is to .
Solubility in alcohol
Dissolve g in 10 ml of boiling alcohol. The solution is not more opalescent than reference suspension and is colourless.
Organic matter
It does not darken on progressive heating to dull redness.
Sulphates
10 ml of solution S diluted to 15 ml with distilled water complies with the limit test  for sulphates (450 ppm).
Heavy metals
12 ml of solution S complies with limit test A for heavy metals (15 ppm).

Propionate citric acid cycle

propionate citric acid cycle

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