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Carnitine biosynthesis is a method for the ... The current consensus theory about the origin of N ε-trimethyllysine in mammals is that mammals utilise lysosomal ...
Carnitine is a quaternary ammonium compound involved in metabolism in most mammals, plants, and some bacteria. [1] [2] [3] [4] In support of energy metabolism ...
In mammals and humans, αKG-dependent dioxygenase have functional roles in biosyntheses (e.g. collagen biosynthesis [14] and L-carnitine biosynthesis [15]), post-translational modifications (e.g. protein hydroxylation [16]), epigenetic regulations (e.g. histone and DNA demethylation [17]), as well as sensors of energy metabolism. [18]
The function of ACC is to regulate the metabolism of fatty acids. When the enzyme is active, the product, malonyl-CoA, is produced which is a building block for new fatty acids and can inhibit the transfer of the fatty acyl group from acyl-CoA to carnitine with carnitine acyltransferase , which inhibits the beta-oxidation of fatty acids in the ...
English: Endogenous biosynthesis of L-Carnitine from protein-bound amino-acids. 日本語: L- ...
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-carnitine is ...
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.
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]