wmackowiak

In Harper's biochemistry there is a sketch of "amphibolic intermediate products made of aminoacids' carbonic backbone". It shows through which different products aminoacids (not per se) are entering citric acid cycle. In case of pyruvate carboxylase deficiency the conversion of pyruvate INTO oxaloacetate is impaired. Therefore, as I understand correctly, all aminoacids, which enter citric acid cycle through pyruvate, would not be used in the process of gluconeogenesis. Of course the whole process would be impaired but for instance Phenyloalanine is entering the citric acid cycle through fumarate. That would mean that there is no need for conversion into oxaloacetate by pyruvate carboxylase but by fumarate and malate dehydrogenase. I don't know if this question makes a lot of sense; it's quite complex and we wanted to take a look from different topics.

Hello everyone, I have a question regarding pyruvate carboxylase deficiency. Namely, I'd like to know which of the aminoacids is most likely to provide glucose production? I'm arguing with my friends about it. One of them says it's alanine but for me that's the one aminoacid that cannot be transformed to oxaloacetate from pyruvate... What do you think? Other ideas were Phenyloalanine, Valine, Arginine and LEucine (but this one is not glucogenic so I would odd this out) I'm really looking forward to hearing some professional voices