Question

1. A complete deficiency of urea cycle enzyme usually causes death soon after birth, but a partial deficiency may be tolerated.

1. Explain why hyperammonemia (high levels of ammonia in the blood) accompanies a urea cycle enzyme deficiency.
2. What dietary adjustments might minimize ammonia toxicity?

Answer 1

following dietary changes can be made in order to reduce the level of the ammonia in the blood of individual with defected urea cycle enzymes -

1 Reduce the intake of the protein in your diet - As ammonia is a by product of the protein digestion so reducing the intake of the protein rich diet will reduce the ammonia in the blood .

2  stop eating the red meat - red meat diet can lead to high increase in the ammonia in the diet .

3 Add more vegetable and fruits in your diet

4 include the probiotics in your diet - adding probiotics in your diet will make your digestion better and also reduce the level of the ammonia in your body

hence by above dietary changes you can reduce the level of the ammonia inyour diet .

Answer - role of the AMPK on the glucose 6 phosphatase and the muscle glut 4

glut 4 are the glucose transporter which allow the transport of the glucose to the muscle cell . in certain conditions the ampk regulate the glucose intake by translocating the GLUT4 transporter on the membrane .

AMPK inhibit the rab gtpase activating protein (AS160)  which increases the activity of Rab family G proteins lead to translocating of the glut 4 to the membrane for glucose uptake.

on the another hand the AMPK cause the inhibition of the glucose 6 phosphatase and inhibit the gluconeogenesis when the cell has the enough energy source .