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Published in 2019 at "Journal of Inherited Metabolic Disease"
DOI: 10.1002/jimd.12076
Abstract: Deficiency of antiquitin (ATQ), an enzyme involved in lysine degradation, is the major cause of vitamin B6‐dependent epilepsy. Accumulation of the potentially neurotoxic α‐aminoadipic semialdehyde (AASA) may contribute to frequently associated developmental delay. AASA is…
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Keywords:
degradation;
dependent epilepsy;
saccharopine pathway;
degradation pathways ... See more keywords
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Published in 2020 at "Frontiers in Plant Science"
DOI: 10.3389/fpls.2020.00587
Abstract: The saccharopine pathway (SACPATH) involves the conversion of lysine into α-aminoadipate by three enzymatic reactions catalyzed by the bifunctional enzyme lysine-ketoglutarate reductase/saccharopine dehydrogenase (LKR/SDH) and the enzyme α-aminoadipate semialdehyde dehydrogenase (AASADH). The LKR domain condenses…
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Keywords:
aminoadipate;
saccharopine pathway;
lysine catabolism;
stress ... See more keywords