What is the role of carnitine acyltransferase I?

Carnitine acyltransferases are a large family of enzymes that play a main role in cellular energy metabolism, i.e. fatty acid oxidation. These enzymes catalyze the reversible exchange of acyl groups (which derive from fatty acids) between coenzyme A and carnitine.

What does carnitine acyltransferase II do?

The CPT2 gene provides instructions for making an enzyme called carnitine palmitoyltransferase 2. This enzyme is essential for fatty acid oxidation, a multistep process that breaks down (metabolizes) fats and converts them into energy.

Where is carnitine acyltransferase found?

outer mitochondrial membrane
The mitochondrion is the site of the β-oxidation pathway that generates acetyl CoA from fatty acids. Fatty acids, as the CoA derivatives, are transported into the mitochondrion by carnitine acyltransferase located in the outer mitochondrial membrane.

What is carnitine metabolism?

Carnitine is a quaternary ammonium compound involved in metabolism in most mammals, plants, and some bacteria. In support of energy metabolism, carnitine transports long-chain fatty acids into mitochondria to be oxidized for energy production, and also participates in removing products of metabolism from cells.

What causes low carnitine levels?

Carnitine deficiency results from inadequate intake of or inability to metabolize the amino acid carnitine. It can cause a heterogeneous group of disorders. Muscle metabolism is impaired, causing myopathy, hypoglycemia, or cardiomyopathy. Infants typically present with hypoglycemic, hypoketotic encephalopathy.

What do you know about acyl carnitine transferase?

Carnitine palmitoyltransferase I (CPT1) also known as carnitine acyltransferase I, CPTI, CAT1, CoA:carnitine acyl transferase (CCAT), or palmitoylCoA transferase I, is a mitochondrial enzyme responsible for the formation of acyl carnitines by catalyzing the transfer of the acyl group of a long-chain fatty acyl-CoA from …

How common is carnitine palmitoyltransferase II deficiency?

CPT II deficiency is a rare disorder. The lethal neonatal form has been described in at least 18 families, while the severe infantile hepatocardiomuscular form has been identified in approximately 30 families. The myopathic form occurs most frequently, with more than 300 reported cases.

What should I eat with CPT2?

Diet Sometimes a low fat, high carbohydrate diet is recommended. Carbohydrates give the body many types of sugar that can be used as energy. In fact, for people needing this treatment, most food in the diet should be carbohydrates (bread, pasta, fruit, vegetables, etc.) and protein (lean meat and low-fat dairy foods).

Does carnitine burn fat?

L-carnitine is best known as a fat burner — but the overall research is mixed. It is unlikely to cause significant weight loss. However, studies support its use for health, brain function and disease prevention. Supplements may also benefit those with lower levels, such as older adults, vegans and vegetarians.

Is carnitine safe to take?

When taken by mouth: L-carnitine is likely safe when taken for up to 12 months. It can cause side effects such as stomach upset, heartburn, diarrhea, and seizures. It can also cause the urine, breath, and sweat to have a “fishy” odor.

Is carnitine deficiency curable?

CDSP is treatable by the daily use of L-carnitine supplements. Without early detection and treatment, an affected child may experience low blood sugar (hypoglycdemia), seizures, heart muscle weakness (cardiomyopathy) that can be life-threatening. Newborn screening is available for CDSP.

What foods are rich in carnitine?

What foods provide carnitine? Animal products like meat, fish, poultry, and milk are the best sources. In general, the redder the meat, the higher its carnitine content. Dairy products contain carnitine primarily in the whey fraction [1,3,5].

What are the names of acyltransferases that transfer carnitine?

Nomenclature. This enzyme belongs to the family of transferases, to be specific those acyltransferases transferring groups other than aminoacyl groups. The systematic name of this enzyme class is acetyl-CoA:carnitine O-acetyltransferase. Other names in common use include acetyl-CoA-carnitine O-acetyltransferase, acetylcarnitine transferase,…

Which is the enzyme responsible for the formation of carnitine?

Carnitine palmitoyltransferase I ( CPT1) also known as carnitine acyltransferase I, CPTI, CAT1, CoA:carnitine acyl transferase ( CCAT ), or palmitoylCoA transferase I, is a mitochondrial enzyme responsible for the formation of acyl carnitines by catalyzing the transfer of the acyl group of a long-chain fatty acyl-CoA from coenzyme A to l-carnitine.

Where does fatty acyl carnitine diffuse from the cytosol?

Fatty acyl–carnitine can diffuse from the cytosol across the porous outer mitochondrial membrane to the intermembrane space, but must utilize CACT to cross the nonporous inner mitochondrial membrane and reach the mitochondrial matrix.

Why is the carnitine palmitoyltransferase necessary for beta oxidation?

The carnitine palmitoyltransferase system is an essential step in the beta-oxidation of long chain fatty acids. This transfer system is necessary because, while fatty acids are activated (in the form of a thioester linkage to coenzyme A) on the outer mitochondrial membrane, the activated fatty acids must be oxidized within the mitochondrial matrix.