Propionic acidemia, Methylmalonic acidem

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Propionic acidemia, Methylmalonic acidem

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macintosh
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04/08/08 - 07:56 AM

How does Propionic acidemia and Methylmalonic acidemia cause secondary hyperammonemia??

new_n_lost
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04/08/08 - 02:04 PM

I had this written down from somewhere that the accumulation of propionyl CoA results in somehow deactivating CPS 1 and also to do something with the glutamine/glutamate ratio. Cant remember where and dont really know how. but since they big guns arent sure of it I m just happy to know the relationship exists

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sigh
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04/09/08 - 11:47 AM

In organic aciduria (propionic, methylmalonic) acidosis is present. In state of acidosis
activity of glutaminase is increased and we have more glutamate production from glutamine. As new_lost wrote" glutamine/glutamate ratio" will decreased.
But increased glutamate shuld activate CPS-1 through NAG....
Somebody HELP me here....

new_n_lost
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04/09/08 - 01:17 PM

sigh wrote:
"As new_lost wrote" glutamine/glutamate ratio" will decreased.
But increased glutamate shuld activate CPS-1 through NAG....
Somebody HELP me here....

i didnt write that i said that it has to do something with the ratio. and nothing else.

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FORUM RULES-- Those who believe in telekinesis, raise my hand. I get enough exercise just by pushing my luck --P4U World.." The pure and simple truth is rarely pure and never simple."

sigh
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04/09/08 - 04:31 PM

I did put only g/g ratio in " "

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