Hyperammonemia in MCAD def.

This forum made possible through the generous support of SDN members, donors, and sponsors. Thank you.
My guess would be increased utilization of amino acids in the TCA.

This is right. Because beta-oxidation is messed up in MCAD deficiency, once your glucose/glyocogen stores get used up you need to get those carbon chains to make more glucose and/or ketone bodies from somewhere (amino acids). That increased metabolism of amino acids means those nitrogen waste groups build up because urea cycle can only go so fast.
 
Top