Question

A child was born with a deficiency in β-ketoacyl-CoA transferase specifically in the brain. What do you think will be the consequences if the child is fasting for several days.

Explain what will be the consequences of this deficiency.

Answer 1

It's a metabolic disease. This enzyme need for the ketone body metabolizing. Ketone body used as a source of energy when glucose level low in body. When the absent of this enzyme the accumulation of the ketone in the body is called keto acidosis. So this caused the by the physical stree, fasting, illness, low carbohydrates diet etc. Then the symptoms arise like rapid breathing, poor feeding, vomit, lethargy.

When long fasting that time glucose not provide energy to body so that that time body fat break down into the ketone for energy source but ketone can't be used up body because of deficiency of this enzyme so that ketone accumulation in blood and urine. This cause the diabetes.