carnitine metabolic process

Definition

Target type: biologicalprocess

The chemical reactions and pathways involving carnitine (hydroxy-trimethyl aminobutyric acid), a compound that participates in the transfer of acyl groups across the inner mitochondrial membrane. [GOC:jl, ISBN:0198506732]

Carnitine metabolism is a crucial process for energy production, particularly in tissues with high energy demands like the heart and skeletal muscles. It involves the transport of long-chain fatty acids (LCFAs) across the mitochondrial membrane, where they are broken down through beta-oxidation to generate ATP. This process begins with the synthesis of L-carnitine from lysine and methionine. L-carnitine is then transported into the mitochondria via carnitine palmitoyltransferase I (CPT1) and carnitine palmitoyltransferase II (CPT2), which catalyze the reversible transfer of LCFAs from coenzyme A (CoA) to L-carnitine. The resulting acylcarnitine is transported into the mitochondrial matrix where CPT2 releases the fatty acyl group. The fatty acyl-CoA is then subjected to beta-oxidation, a process that cleaves two-carbon units from the fatty acid chain, generating acetyl-CoA and reducing equivalents (NADH and FADH2) that drive ATP production through oxidative phosphorylation. The cycle is completed with the release of carnitine from the mitochondria and its recycling for further transport of LCFAs. Deficiencies in carnitine metabolism can lead to various metabolic disorders, affecting energy production and potentially causing muscle weakness, cardiomyopathy, and other health complications. '
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Proteins (4)

Compounds (17)