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The second step of L-carnitine biosynthesis requires the 3-hydroxy-N ε-trimethyllysine aldolase enzyme. 3-hydroxy-N ε-trimethyllysine aldolase is a pyridoxal phosphate dependent aldolase, and it catalyses the cleavage of 3-hydroxy-N ε-trimethyllysine into 4-N-trimethylaminobutyraldehyde and glycine.
The form present in the body is l-carnitine, which is also the form present in food. Food sources rich in l-carnitine are animal products, particularly beef and pork. [1] Red meats tend to have higher levels of l-carnitine. [1] [24] Adults eating diverse diets that contain animal products attain about 23–135 mg of carnitine per day.
Gamma-butyrobetaine dioxygenase (also known as BBOX, GBBH or γ-butyrobetaine hydroxylase) is an enzyme that in humans is encoded by the BBOX1 gene. [5] [6] Gamma-butyrobetaine dioxygenase catalyses the formation of L-carnitine from gamma-butyrobetaine, the last step in the L-carnitine biosynthesis pathway. [7]
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.
Acyl-carnitine is shuttled inside by a carnitine-acylcarnitine translocase, as a carnitine is shuttled outside. Acyl-carnitine is converted back to acyl-CoA by carnitine palmitoyltransferase II, located on the interior face of the inner mitochondrial membrane. The liberated carnitine is shuttled back to the cytosol, as an acyl-CoA is shuttled ...
Carnitine O-acetyltransferase also called carnitine acetyltransferase (CRAT, or CAT) [5] (EC 2.3.1.7) is an enzyme that encoded by the CRAT gene that catalyzes the chemical reaction. acetyl-CoA + carnitine CoA + acetylcarnitine. where the acetyl group displaces the hydrogen atom in the central hydroxyl group of carnitine.
Acetyl-CoA carboxylase (ACC) is a biotin-dependent enzyme (EC 6.4.1.2) that catalyzes the irreversible carboxylation of acetyl-CoA to produce malonyl-CoA through its two catalytic activities, biotin carboxylase (BC) and carboxyltransferase (CT).