, enumerate the molecular basis of marple syrup disease, health and medicine homework help

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in a 3 page paper, enumerate the molecular basis of marple syrup disease. APA and use medical language please.

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Ketogenesis

5/25/2017

SKG

BIOC116

1

Introduction


During high rates of fatty acid oxidation,
primarily in the liver, large amounts of acetylCoA are generated.



These exceed the capacity of the TCA cycle,
and one result is the synthesis of ketone bodies,
or ketogenesis.

5/25/2017

SKG

BIOC116

2



The ketone bodies are
Acetoacetate,
 β-hydroxybutyrate, and
 Acetone.


5/25/2017

SKG

BIOC116

3

Acetoacetate






The formation of acetoacetyl-CoA occurs by
condensation of two moles of acetyl-CoA through a
reversal of the thiolase catalyzed reaction of fat
oxidation.
Acetoacetyl-CoA and an additional acetyl-CoA are
conve...


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