Lysine degradation pathway

The lysine degradation pathway refers to the sequence of biochemical reactions leading to the breakdown of the essential amino acid lysine. In humans and most mammals, lysine is mainly degraded by the saccharopine pathway, which starts in the mitochondria with the enzyme alpha-aminoadipic semialdehyde synthase (AASS), a bifunctional enzyme comprising lysine-2-oxoglutarate reductase (LOR) and saccharopine dehydrogenase (SDH) domains[2][5][6][8]. This pathway is clinically relevant because deficiencies in various enzymes result in inborn metabolic errors, including glutaric aciduria type 1 and pyridoxine-dependent epilepsy[8]. There is an alternative pipecolic acid pathway primarily in the brain[6][8]. The pathway's intermediates and products can serve as diagnostic biomarkers for related metabolic disorders. While the pathway itself is not a single target, enzymes within it, such as AASS, can be considered valid therapeutic targets in the context of rare metabolic diseases[2][8].

Other names

Lysine catabolic pathwayLysine catabolism