help please - propionic acidemia

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help please - propionic acidemia

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Tiff
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03/25/08 - 05:06 PM

Can anyone explain why treatment for propionic acidemia (deficiency of propionyl coA carboxylase) involves supplementing methionine, valine, isoleucine and odd-chain fatty acids? Why supplement with these when these when metabolized produce propionyl CoA? raised eyebrow

Seems useless and counterproductive. confused

Anyone can explain?

Kalyann
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Topics: 4
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03/25/08 - 05:10 PM

Rx involves restricing them from diet

and may be they r essential amino acids... a small amount is reqd for normal bodili functions

lets c others replies tooo

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new_n_lost
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03/25/08 - 09:36 PM

I thought the Rx was to totally stop protein from diet till the acidemia resolves and then instituting essential amino acids under strict supervision along with Biotin & L-carnitine supplementation.

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Tiff
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03/25/08 - 10:41 PM

new_n_lost wrote:
I thought the Rx was to totally stop protein from diet till the acidemia resolves and then instituting essential amino acids under strict supervision along with Biotin & L-carnitine supplementation.

Agree. Essesntial amino acids being isoleucine, methionine, valine. But why? Wouldn't this produce more propionyl CoA? Doesn't seem like it's helping the problem.

new_n_lost
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03/26/08 - 12:08 AM

well how would you survive without those " Essential Amino Acids" wink

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Tiff
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03/26/08 - 05:50 PM

Haha.

But these patients are not deficient right? And giving more looks like there'd be increased propionyl coA. Really must be missing something here. sad

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