刘新文,脂类代谢 )饱和脂肪酸的氧化 1.脂肪酸的活化内质网,线粒体外膜 RCOOH CoAsH + ATP 脂酰CoA合成酶 RCOW SCOA+AMP+ PPi 2.脂酰CoA的转移 CH3 肉碱:B羟γ三甲基氨基丁酸( CH2-N+-CH-CH-CH:coo carnitine CH2 oh
刘新文，脂类代谢 （一）饱和脂肪酸的氧化 1. 脂肪酸的活化 内质网，线粒体外膜 RCOOH + CoASH + ATP RCO SCoA + AMP + PPi 2. 脂酰CoA的转移 Mg2+ 脂酰CoA合成酶 肉碱：β羟γ三甲基氨基丁酸 carnitine CH3 | CH3-N+-CH2-CH-CH2-COOH | | CH3 OH
刘新文,脂类代谢 0.5m dNA Crista Matrix Ribosomes Inner membrane Outer membrane Figure 2-15 Structure of a mitochondrion. This
刘新文，脂类代谢
刘新文,脂类代谢 脂酰CoA+肉碱 COASH+脂酰肉碱 线粒体外 线粒体内膜线粒体内 脂肪酸→脂酰~SCoA 肉碱↑脂酰~SCoA 酶I 脂酰 COASH 肉碱 COASH 肉碱脂酰转移酶I:限速酶 肉碱脂酰转移酶ⅠI 肉碱:肝赖氨酸;肾
刘新文，脂类代谢 线粒体外 线粒体内膜 线粒体内 脂肪酸 脂酰 SCoA CoASH 肉碱 脂酰 肉碱 脂酰 SCoA CoASH 酶I 酶II 肉碱脂酰转移酶I： 限速酶 肉碱：肝 赖氨酸； 肾 肉碱脂酰转移酶II: 脂酰CoA + 肉碱 CoASH + 脂酰肉碱
刘新文,脂类代谢 3.脂肪酸的β氧化β- oxidation 线粒体 RCH-CHb-CH-CO SCoA 脂酰CoA/FAD 继续β氧化 脱氢酶N FADH2 RCHCH=CH-CON SCOA RCH -CON SCoA+ CHr-CON SCoA 烯酰CoA HO β脂酰CoA 水合酶 COASH β羟脂酰 硫解酶 RCH2CHCH2 CON SCoA COA脱氢酶RCH2 -C-CH2 COn SCoA OH NAD+ NADH +H O
刘新文，脂类代谢 3. 脂肪酸的β 氧化 β-oxidation 线粒体 RCH2-CH2-CH2-CO SCoA RCH2CH=CH-CO SCoA 脂酰CoA 脱氢酶 FAD FADH2 RCH2-C-CH2-CO SCoA || O β羟脂酰 CoA脱氢酶 NAD+ NADH + H+ 烯酰CoA H2O 水合酶 RCH2-CH-CH2-CO SCoA | OH β脂酰CoA CoASH 硫解酶 RCH2-CO ~ SCoA + CH3-CO ~ SCoA 继续β氧化
D CH3 一COo CoASH NAD' 类代谢 cO,+ NADH (Acetyl-CoA)CH,-C-S-CoA Coo H,O- 'cOO CHe .coO HO-C-COo v’coo .coO Citrate HO-C-H NAD 8 malate hydrogenate coo Watcom H HO-C-H 7. fumarase CItric acid CH Watcom H dehydrogenase FAD hydrogenate 2C00 5. succinyl-CoA COASH .coo NAD y2'coo GDPHP CoA Succinyl CoA Figure 16-2. Key to Metabolism. The reactions of the citric becomes Cl of a-ketoglutarate and is released as CO, in Reaction cyele. The reactants and products of this catalytic cycle are 4. An isotopic label at Cl of acetyl-CoA (t) becomes C5 of a-ketoglutarate and is scrambled in Reaction 5 between Cl and s substrate via carbohydrate metabolism but is not considered C4 of succinate (h1) part of the cycle. An isotopic label at C4 of oxaloacetate (
刘新文，脂类代谢