What does acetyl CoA dehydrogenase do?
Acyl-CoA dehydrogenases (ACADs) are a class of enzymes that function to catalyze the initial step in each cycle of fatty acid β-oxidation in the mitochondria of cells.
What is the effect of the lack of acyl-CoA Dehydrogenase?
Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is an inherited disorder that prevents your body from breaking down certain fats and converting them into energy. As a result, the level of sugar in your blood can drop dangerously low (hypoglycemia).
What is Vlcad deficiency?
Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency is a condition that prevents the body from converting certain fats to energy, particularly during periods without food (fasting).
How should a patient with a medium-chain acyl-CoA dehydrogenase deficiency be treated?
Strategies may include:
- Taking simple carbohydrates, such as sugar (glucose) tablets or sweetened, nondiet beverages.
- Seeking emergency medical care if unable to eat or for diarrhea or vomiting. An intravenous (IV) line for extra glucose and additional treatment may be needed.
Is MCADD a disease?
MCADD is a rare genetic condition where a person has problems breaking down fat to use as an energy source. This means that someone with MCADD can become very ill if their body’s energy demands exceed their energy intake, such as during infections or vomiting illnesses when they’re unable to eat.
Is VLCAD curable?
If treated early, babies with very long-chain acyl-CoA dehydrogenase deficiency (VLCAD) can have healthy growth and development. Treatments need to continue throughout life. If VLCAD is not treated, babies usually die young. It is important to screen for and treat VLCAD.
What are two common treatments for VLCAD deficiency?
Treatment. Management of VLCAD deficiency is focused primarily on preventing acute episodes of low blood sugar (hypoglycemia). This process includes avoiding fasting and using a very low-fat, high-carbohydrate diet, with frequent feeding.
