Dienoyl-CoA+reductase

=__**Dienoyl-CoA Reductase**__= __(__Dienoyl-CoA reductase deficiency **OR** 2,4-alpha dienoyl-CoA reductase deficiency **OR** DECR1)



__What?__
It's a protein which is encoded by the DECR1 gene that is located on chromosome 8. The gene codes for an extra enzyme that participates in the beta oxidation and metabolism of unsaturated fats. //Basically, it is a fatty acid metabolism disorder.//

**__How?__** This disorder usually follows a recessive inheritance pattern. In this case, affected patients usually have two copies of a mutation in order to show symptoms of the disease such as;
 * early death
 * feeding difficulties, poor feeding
 * hypotonia
 * respiratory acidosis
 * vomiting

__Treatment?__
It's suggested to feed the patient a formula containing fat derived from medium-chain triglycerides (MCT) which distributes pharmacological doses of carnitine. However, due to the complexity of this disorder, the patient must be observed be their doctor and a pediatric metabolic disease specialist.