Harpers

OOCCH 2CH 2COCO –H 2 OOCCH 2CH 2COCO –NADH+ H + NAD +OCCH 2CH 2CHOCO –– OH 2 C– OSaccharopineα-Ketoglutarate+NH +NH 3+NH 2+NH 3+– OL-LysineH 2NH+NH +3H 2 C CH 2 CHCH 2 CH 2 CO –H 2 CCH 2CH 2CH 2CHCOO –1,2SACCHAROPINEDEHYDROGENASE,L-LYSINE-FORMINGCH 2CH 2CH 2CHCOO –OOO256NAD +2NADH+ H +SACCHAROPINEDEHYDROGENASE,L-GLUTAMATE-FORMING– OOCO –2OCHCHCH 2 CNH + NH+ 3HCCH 2CH 2CH 2CHCOO –H 2 O– OOHCCCH 2CH 2CHL-GlutamateNH 3+NH 3+CH 2CH 2CH 2CHCCO –NAD + NADH + H + ONH+ 3C CH– 2 CHO CH 2 CH2 CH 2 OOL-α-AminoadipateO –O –OL-α-Aminoadipateδ-semialdehydeα-KG Glu OOCoASHPLPTRANSAMINASEC CH 2 C O –– O CH 2 CH 2 Cα-KetoadipateO[O]CO 2OC CH 2 C– O CH 2 CH2Glutaryl-CoAOSCoACO 2 + H 2 OFigure 30–14. Catabolism of L-lysine. (α-KG, α-ketoglutarate; Glu, glutamate; PLP, pyridoxal phosphate.) Circled numerals indicate the probable sitesof the metabolic defects in 1 periodic hyperlysinemia with associated hyperammonemia; and 2 persistent hyperlysinemia without associated hyperammonemia.