Pathways
Lysine Degradation in Human Liver
Description:
In humans, Lysine is an essential amino acid, and there is no Lysine biosynthetic machinery. However, humans do degrade Lysine. Lysine is incorporated to Collagen, one of the most important components of Connective tissue and its supply is therefore required during embryonic development and early childhood (Ref.1). It is also important for Carnitine Synthesis. The main catabolic pathway for Lysine, via Saccharopine (e-N-(L-Glutaryl-2)-L-lysine), is a mitochondrial pathway leading to the formati...
References:
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Simultaneous retention index analysis of urinary amino acids and carboxylic acids for graphic recognition of abnormal state.
J. Chromatogr. B. Analyt. Technol. Biomed. Life Sci. 2005 May 12. -
Characterization of the human gene encoding alpha-aminoadipate aminotransferase (AADAT).
Mol. Genet. Metab. 2002 Jul;76(3):172-80. -
New insights into human lysine degradation pathways with relevance to pyridoxine-dependent epilepsy due to antiquitin deficiency.
J Inherit Metab Dis. 2019 Jul;42(4):620-628. doi: 10.1002/jimd.12076. Epub 2019 Apr 15 -
Identification of the alpha-aminoadipic semialdehyde synthase gene, which is defective in familial hyperlysinemia.
Am. J. Hum. Genet. 2000 Jun;66(6):1736-43.
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