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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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.
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.
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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