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Topic: why is ketosis seen in organic acidemias  (Read 4368 times)

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Offline rosegarden1

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why is ketosis seen in organic acidemias
« on: November 01, 2015, 04:05:13 PM »
Maybe the questoin is simple but i couldn't find an answer in clinical textbooks. organic acidemias are related with amino acid catabolism and there's a block in catabolism pathway. so if the amino acids can't degrade to their end products such as pyruvate or acetyl coA, where the ketone bodies seen in these disorders come from?

Offline ATMyller

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Re: why is ketosis seen in organic acidemias
« Reply #1 on: November 02, 2015, 04:13:08 AM »
Beta-oxidation of fatty acids produces acetyl-CoA as well.
Chemists do it periodically on table.

Offline rosegarden1

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Re: why is ketosis seen in organic acidemias
« Reply #2 on: November 02, 2015, 04:38:37 AM »
why does the beta oxidation of fatty acid increase then?

Offline Babcock_Hall

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Re: why is ketosis seen in organic acidemias
« Reply #3 on: November 02, 2015, 08:23:06 AM »
Do you have a citation which indicates that the production of ketone bodies rises when there is a particular metabolic block?  I have to wonder whether or not we may be thinking of two different things.  in phenylketonuria, the concentrations of phenylpyruvate and related metabolites rise in the urine and blood, for example, and phenylpyruvate is an organic ketoacid.
« Last Edit: November 02, 2015, 10:30:01 AM by Babcock_Hall »

Offline rosegarden1

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Re: why is ketosis seen in organic acidemias
« Reply #4 on: November 02, 2015, 12:03:09 PM »
luckily i found the answer. thank you for your reply.
organic acids can inhibit gluconeogenesis therefore fatty acid oxidation increases to meet body's energy requirement. Also organic acidemias cause hyperammonemia by inhibiting urea cycle.

reference:https://books.google.com.tr/books?id=a7CqcE1ZrFkC&pg=RA3-PA987&dq=organic+acidemia+ketosis&hl=tr&sa=X&redir_esc=y#v=onepage&q=organic%20acidemia%20ketosis&f=false

Offline Babcock_Hall

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Re: why is ketosis seen in organic acidemias
« Reply #5 on: November 02, 2015, 05:29:28 PM »
Your reference also notes that some organic acidemias are associated with hypoketotic hyperglycemia.  I am not surprised that difference metabolic defects show a variety of outputs.

Offline rosegarden1

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Re: why is ketosis seen in organic acidemias
« Reply #6 on: November 03, 2015, 01:02:31 AM »
yep,that's related with which organic acid is accumulated.

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