87.下列有關葡萄糖氧化的五碳糖磷酸途徑(pentose phosphate pathway)功能之敘迹,何者正確?
(A)負責催化每莫耳葡萄糖產生36莫耳ATP
(B)負責催化每莫耳葡萄糖産生5莫耳CO2
(C)供應合成核苷酸(ucleotide)所需要之核糖(ribose)的來源
(D)主要功能是產生NADH及FADH2進入呼吸鏈氧化産生能量
統計: A(14), B(5), C(776), D(57), E(0) #2408131
詳解 (共 3 筆)
-
PPP: another metabolic pathway from G6P to R5P and NADPH, except for glycolysis
-
分成oxidative (生R5P+2NADPH) and nonoxidative (生G6P)
(1) Oxidative (irreversible): 透過glucose 6-phosphate dehydrogenase (rate limiting enzyme)將G6P (羥基)轉為lactone (酮),再透過lactonase轉為gluconate (酸),再透過6-phosphogluconatedehydrogenase轉為ribulose 5-phosphate+CO2。
(2) 病:Favism (缺glucose 6-phosphate dehydrogenase)缺NADPH還原glutathione (GSSG --glutathione reductase--> 2 GSH),GSH可以還原peroxides/reactive oxygen species。吃fava beans造成氧化壓力,RBC無保護蛋白,因氧化壓力而lyse (hemolytic anemia)。



(3) 調節:NADPH會inhibit PPP (step: G6P -> lactone)
(4) 特殊:當更需要NADPH而非ribose,可以產生12NADPH。
(5) 適用地點:
A. Ribose: rapidly dividing cells (BM, skin, intestinal mucosa, tumor)
B. NADPH: fatty acid, cholesterol and steroid hormones synthesis (liver, adrenal gland腎上腺, gonad 性腺)、O2 exposure (RBC, lens and cornea 角膜)
-
Nonoxidative (reversible): 透過transketolase (轉移C2 unit) 與transaldolase (轉移C3 unit),生成F6P (->G6P)。
(1) TPP: vitamin B1在細胞內形式,作為coenzyme幫助轉移C2 unit、stabilize carbanion intermediate
(2) 病:Beriberi (缺vitamin B1)、Wernicke-Korsakoff syndrome (transketolase mutation, less affinity to TPP)
(3)Lys: transaldolase 側鏈N的lone pair與C2 unit共振 (l.p.作為路易斯base,親和攻擊隔壁碳,stabilize carbanion intermediate)