生物化学课件英文版教授讲授下册习题答案

1、EXERCISESCHAPTER171.(1) ATP 水解的G0 7.3(kcal/mol), creatineosate 水解的G010.3, 两者偶联的G0为 3,反应向左。3.（a）总的G0 7.5(kcal/mol): 7.3 (14.8) = 7.5平衡时G=0； G=G0 +1.364 lg Keqlg Keq = G0/1.364 = 5.5;Keq = 3.06 x 106(b) Keq = PEP / ATPPyr,ATP / = 10,Pyr / PEP= 3.28 x 1044. 将 G-6-P（3.3）和 G-1-P（5.0）水解的G0叠加（偶联）(= 1.7)然后计

2、算 Keq。（lg Keq = G0/1.364=1.25; Keq = 17.8G-1-P/ G-6-P） G-6-P G-1-P+ PPi）和 acetyl CoA 水解的G0（见 P451）各5. (a) ATP（为7.3 和7.5；总的G0为 0.2（b）Acee + ATP +CoA acetyl CoA + PPi (2 Pi)再与 PPi 的水解偶联 0.28.0 = 7.8焦磷酸水解推动反应朝着形成 acetyl CoA 的方向进行.这是生物化学中的一个普遍原则:生化反应经常与焦磷酸水解相偶联,从而推动反应朝着正反应的方向进行(使原本热力学上不可行的反应成为可行). 如 R-C

3、OO + CoA + ATP acyl CoA +PPi6. G=G0 +2.303RT lg Keq;G0 = 2.303RT lg Keq = 2.303RTG0 = 1.364; (1.364 x值小G0 值小4.8= 6.53)酸性强质子转移势大Keq值大CHAPTER193. (a)G0=(4.0+ 0.43.4+ 5.7+ 1.8)+ 2x(1.54.5+ 1.1+ 0.47.5)+ 2x(6)= 29.5(b) Glc + 2Pi+ 2P4912 Lac +2ATP+ 2H2O P490, P497 G=G0+ 1.364 lgATP2Lac2/GlcPi22K=(2x10-3)

4、2(0.05x10-3)2/(5x10-3)(0.2x10-3)2(10-3)2=200G=29.5 + 1.364 lg200 = 27.2 kcal/molG=G0+ 1.364 lgKeq4. PEP+Pyr+ ATP平衡时, G=0,可从表中查出G0值,即可计算出KeqPEP /Pyr=3.06 x 105Keq = PyrATP/PEP“很高的转移势”指 PEP(即使在PEP /Pyr很小时)能将磷生成 ATP酸基转移给5. F-1,6-BPGAl-3-P +DHAPaldolase(醇)醛缩(合)酶G=G0+ 1.364 lgKeqG0 Keq x0.001xxxKeq= x2/0

5、.001x8（a）1，3-BPG2,3BPGoxygen affinity of Hb高(低)水平糖酵解产物导致血红蛋白氧气亲和力的降低(增强)（b）己糖激酶缺陷,即糖酵解受阻糖酵解中间物缺乏即 2,3BPG血红蛋白氧气亲和力（c）酸激酶缺陷,糖酵解出口受阻糖酵解中间物血红蛋白氧气亲和力9. F-1-P 途径(直接)形成 Gal-3-P 绕过 PFK(使得过多的酵解产物不能有效抑制PFK), F-1-P(见P491)还刺激生成过多乳酸;.过多乳酸生成导致酸12.（a）PFK 的 ATP 变构位点丢失能量充足信号不能抑制糖酵解糖酵解步伐PFK 的柠檬酸结合位点丢失糖酵解产物(乙酰辅酶 A) 充足

6、信号不能抑制糖酵解糖酵解步伐调控 F-2,6-BP 水平的双功能酶中磷酸酶结构域丢失 磷酸化功能F-2,6-BP 形成糖酵解步伐（d酸激酶()中 F-1,6-BP 结合位点失糖酵解前体步骤标志物充足信号不能刺激CP CC糖酵解步伐C C C C C C P(G-6-P)C C C P(F-1,6-BP)C P C C(DHAP)C CC PCC C P(GAl-3-P)+(GAl-3-P)C P C C P(1,3-BPG)C CC P(3-PGA)CC PCC C P C(PEP)COOH C=O CH3(Pyr)(2-PGA)（糖酵解：葡萄糖标记的一号碳原子出现在酸的甲基碳中）COOCOO

7、COOCOOCH3+C=O C=OCH2CH2CH2HOCCOOCH2 COOHCCOOHCOH COOCH2COO-CH2+CO2C=O COO(OAA)(acetyl CoA)(citrate)(isocitrate)(-KG)COOCH2CH2 COO+CO2(succinyl CoA)(上述酸标记的甲基碳经过氧化脱羧出现在乙酰辅酶 A乙酰基的甲基碳中，经 TCA 循环的前四步，此标记的碳原子出现在二羧酸的 2,3 碳原子中)CHAPTER201.(a)Pyr 中的甲基碳(b)Pyr 中的羧基碳Pyr 中的羰基碳(d)acetyl CoA 甲基碳(e)G-6-P 中的1 号碳a,d,e

8、甲基碳经过一轮柠檬酸循环出现在 OAA 的 C-2(C-3)中.2.(a)Isocitratelyase(6=4+2)andmalatesynenzymes of citric acid cyclese(2+2=4)+(b)one round of glyoxylate: 2acetyl CoA + NAD+ + 2H2O succinate + 2CoA + NADH + 2H+from succinate to OAA in one round of citric acid cycle:succinate + H2O + FAD + NAD+ OAA + FADH2 + NADH+H+)

9、2acetyl CoA + 2NAD+ + FAD + 3H2OOAA + 2CoA +2NADH + FADH2 + 3H+(ADH)4. CH3-CH2-OH + NAD+ CH3CHO +NADH + H+(Gal-3-P DH)1,3-BPG + NADH + H+ Gal-3-P(no H) + NAD+ + Pi(NAD+内含一个 H)deuterated:氘标记的结论:脱氢酶与辅酶(NAD+或 NADH)的结合有两种不同的方式:ADH 醇脱氢酶(和lac DH 乳酸脱氢酶)与辅酶(NAD+)结合的特异性是A 型的(即A 位朝外)而Gal-3-P DH 三磷酸甘油醛脱氢酶(和 ma

10、late DH 蘋果酸脱氢酶)与辅酶(NAD+)结合特异性是 B 型的(即 B 位朝外,环平面旋转了 180 度)的(见图 20-16,酰胺环中, 酰胺基书写在右侧,则 C4 连接氢原子有两个不同的位点:朝平面外(内)的为A(B)位; C4 连接一(二)个氢原子时为 NAD+(NADH). 5.thiazolone 噻唑酮6.malate + NAD+OAA + NADH 令MALATE/OAA =xG =G0 + 1.364 lgOAANADH/MALATE NAD+7= 1.364 lg 1/8xx=1.75 x 104the value x must be greatern 1.75 x

11、 104for OAA to be formed结论:在稳态条件下OAA是以催化量(即极低浓度)存在的.9. Dj vu 似曾相识;和水解 F-2,6-BP 的双功能酶与(其激酶和磷酸酶的活性部位在不同的区域)相反,修饰异柠檬酸脱氢酶的双功能酶(P524)用同一个活性中心催化相反的反应.CHAPTER211. (b)LacPyr (+1 NADH)(12.5+2.5=15 or 12.5+1.5=14)(c) GlcG-6-P(1)F-6-PF-1,6-BP(1)(30+1+1=32)(d)PEPPyr (+1)(12.5+1=13.5)(e)Gal 半乳糖+ATPGal-1-P(1) +UD

12、P-GlcG-1-P厂G-6-P(311=30)(f)DHAP 磷酸二羟基 GAl-3-P 三磷酸甘油醛1,3-BPG(+1 NADH)3-PGA(+1)2-PG(12.5+2.5+1+1=17 or 12.5+1.5+1+1=16)PPyr(+1)能的改变G0(化2.标准氧还电位(差) E0标准学反应的)平衡常数Keq(规定)氢离子浓度(的差异)E0=G0/nF=(RT ln Keq)/nF =2.3 RT lg Keq /nF = 2.3 x 1.987 x 103 x 298 lg Keq/23.063n=(1.364/23.063n ) lgKeq = (0.059/n )lg Keq

13、E0=(0.059/n) lg Keq或由 Nernst 方程：E01= E0+ (0.059/n) lgox1/red (生物化学)E02= E0+ (0.059/n) lgox2/red (普通化学)2H+ +2eH2(Keq=ox/red), n=2E0= E01E02=0.059/n lg (107)2lg (100)2=0.059=0.42V(H+=107 M;H+=1M)结论：每增加 1 个, E 值减少 0.059V化学教科书上( =0)2H+/H2: 0V;O2/H2O:1.23V生化教科书上( =7)2H+/H2:0.42V; O2/H2O:0.82VPi/O 磷氧比(ind

14、ex of OP)2e + 2H+ + 1/2O2 = H2O4P:O每消耗一个氧原子(即传递一对电子)掺入到有机 ATP中的无机磷酸数(即消耗的无机磷酸数) （NADH：2.5;FADH2: 1.5）(a) H+(translocated)/2e 每传递一对电子被泵出的质子数质电比 (NADH:10;FADH2: 6)P/ H+每一个 ATP 并将它转移到胞质所需质子数(的倒数)磷质比(1/4)5. thermodynamic constra热力学限制E0 for Fum./succi.= 0.03V;E0 for NAD+/NADH=0.32Vsucci+ NAD+Fum. + NADHE

15、0 for FAD/FADH2=0VE=0.32V0.03V=0.35V(以 NAD+作为氧化剂)E=0V0.03V=0.03V(以 FAD 作为氧化剂) G0=nFE =46x(0.35)= 16.1 kcal/mol (以 NAD+作为氧化剂)G0=nFE=46(0.03)=1.4 kcal/mol(以 FAD 作为氧化剂)结论:琥珀酸被 NAD+氧化(成延胡索酸)在热力学上不可行;而被 FAD 氧化(成延胡索酸)在热力学上可行.6.cyanide antidote化物解毒剂nitrite 亚硝酸盐化物与细胞色素氧化酶中 Fe3+相结合(阻断氧化磷酸化)而致死; 亚硝酸盐可将血红蛋白中的

16、Fe2+氧化成 Fe3+,后者与化物结合.即铁血红蛋白可与细胞色素氧化酶争夺化物.且前者的(不损害载氧功能的可用)量远大于后者(故可用于治疗).8. G =nFE=G0 +2.3 RT lg KeqE=0.2V,so G =n x 23 x 0.2=4.6 n kcal/mol+ Pi = ATP + H2OKeq= ATP H2O / PiA. n=2 G =4.6x2=9.2 kcal/mol9.2=7.3 +1.364lg KeqKeq=101.9/1.364=26.213.8=7.3 +1.364lg KeqKeq=106.5/1.364=6.51x104B. n=3(线粒体悬浮液AT

17、P 一直达到这一比值的程度)C. n=4结论:Keq=1011.2/1.364=1.62x108一个(摩尔)ATP 消耗的能粒体悬浮液中每量约 13 千卡,即需移位约 3 个质子(而不是 7.3 千卡,即不是移位约 2 个质子)10. dicyclohexylcarbodiimideDCCI 二环己基亚胺CHAPTER221. C6*P-C-C-C-C-C1(G-6-P)C5*P-C-C-C-C1 (Rib-5-P) +CO22. 6-osogluconate ( -ketoacid葡萄糖酮酸)Rib-5-P +CO2isocitrate (oxalosuccinate 草酰琥珀酸) -KG

18、+CO2脱氢 + 脱羧3. Rib-5-P:C5P-C-C-C-C1* (Xyl-5-P) + C5P-C-C-C-C1* (R-5-P)C3P-C-C1 (GAl-3-P) + C7P-C-C-C-C*-C-C1*(Sed-7-P)C4P-C-C-C1 (Ery-4-P) + Cp(6)-C-C-C*-C-C* (1) (F-6-P)6. TA+ substrate Schiff base + NaBH*4 Lys*(in TA )labeledfingrtritiated 氚标记的H +H+2GSH+N+7.GSSG+NGSSG+2e2GSH(1)H +H+E0=0.23V+N+2eN(2

19、)E0=0.32V(1) (2) E0=0.09VG0=nFE0 = 46x 0.094.15 kcal/mol=G=G0+ 1.364 lg KeqKeq=104.15/1.364=1126Keq=GSH2N+/GSSG NH/ NH= GSH2/GSSGx+)1/X(X=NX=1/ Keq x GSH2/GSSG=1/1126 x (10)2/1=8.9 x 102 (?) X=1/ Keq x GSH2/GSSG=1/1126 x (102)2/103=8.9 x 1058. (because of F-1,6-BPase deficiency 此酶对 F-2,6-BP 调控不敏感) G

20、lc（GNG）produce heat (substrate cycling),F-1,6-BPPyracetyl CoAATP9.biotin + Lys-Pyr carboxylase carrier of activated CO2 (fromHCO3 +ATP to Pyr)lipoic acid +Lys-dihydrolipoyl acetyltransferas（e E2）carrierof acetyl group (from TPP to CoA)10. Avidin 亲和素, 抗生物素蛋白Chapter 23 习题1.G-1-P+UTP+glycogenn+H2Oglyco

21、genn+1+UDP+2Pi (P588:(2)(4) )+H+Gal+ATP G-1-P+(P492)Total:Gal+UTP+ATP+glycogenn+H2O glycogenn+1+UDP+ 2Pi+H+2. glycogen*+(Pi+E) G-1-P/Glc=100 (该glycogen+(Pi+E) G-1-P/Glc=10 (正常人)cause: branching enzyme deficiencymuch little branching的分枝酶缺陷,导致该由于糖原糖原时分枝比正常人少十倍3.(P598)G-6-Pase(ortransporter) G-6-P (oso

22、rylated glycogen synse ba) glycogen5.(P585,503)G-1,6-BP(fromG-1-P+ATP)makestheutase (notosorylated)osorylated.8.(a)P590无活性 Eb-有活性 Ebdegradation变构效应物不起作用，只有可逆磷酸化才能使磷酸化酶成为 Ea型(b)P590Eb-Eadegradation肝糖原动用受阻(c)kinase 过度表达osorylation of Eactivation ofEdegradationlittle glycogen present(d)P596loss of the

23、geneinhibitor of PP1PP1osorylase aor bdegradation(e) loss of te of G subunit of PP1PP1去磷酸化能力大大下降 (P596)syndegradationse andosorylase(f)glycogeninblock the initiation of glycogen synthesisChapter24习题+NADH+H+ (P606)ate DH)1. (A)glycerol+ATP+NAD+DHAP+(glycerol kinase and glycerol(B)DHAP(GAl-3-P)+NAD+2o

24、s+PiPyr+NADH+H+2ATP+ H2OTotal: glycerol+ 2NAD+2H+ + H2O2. (A) stearate 硬脂酸+ATP+CoAPPi+ H2O2Pi+PiPyr+ ATP+ 2NADHstearyl-CoA+(P607)+PPi(B) stearyl-CoA+8FAD+8 NAD+8CoA+8 H2O9acetylCoA+8FADH2+8NADH+8H+(P611)(C) 2acetylCoA+ H2Oacetoacee+2CoA (4.5)(P612)stearate+ATP+13.5H2O+8FAD+8NAD+total:e+14.5H+8FADH2+

25、8NADH+4.5acetoace+2Pi4.palmitoleate 棕榈油酸（16:1 cis9） -7oleate 油酸（18:1 cis9）-9(7 可以自身)linoleate 亚油酸（18:2 cis912）-6linolenate 亚麻酸（18:3 cis91215）-3 (哺乳动物没有这样的酶:可以将双键引入脂肪酸的第九以上的碳原子.7 只能从必需脂肪酸获得,并以此为基础再延长和去饱和)5. CH3(CH2)nCOOHCH3(CH2)n+1 CH2COOHCH3(CH2)n+1CH2CH2 CH2COOH方向由甲基到羧基,即最后加入的二碳辅酶 A 提供)出现在羧基端.丙二酰辅酶

26、A 三个碳原子都 14C 标记加入到的细胞提取液中一分钟后改变此提取液的(由丙二酰脂肪酸使反应突然中断(假定含放射性C 多?一个棕榈酸约需),C-1 还是 C-146. see “aldolase form a Schiase with DHAP” (P500); enolate烯醇化. 一个硫酯的烯醇化阴离子()进攻另一硫酯的羰基碳原子(+)形成CC 键7. (Pyr)OAP (+CO2) in gluconeogenesis 糖异生8. surfeit 过量 promotor muionprotein kinaseA (adie cell)lipase(byosorylation) (P6

27、06 line4)breakdown of TAGand depletion ot stores 级联放大系统通过使脂(肪)酶磷酸化(促活它),从而促进脂肪降解9. Ser in acetyl CoA carboxylase (theof protein kinase) ismuedo Alaactivity of acetyl CoA carboxylase(notinhibited byosorylation 原本是受磷酸化抑制的) FAsynthesisobesity 肥胖10. blocked assets 被阻断的Carnitine 肉碱 translocase 移位酶 defici

28、encyimpairedtransport of acyl from cytosolo Mito (FA)G-6-P transporter deficiencyimpaired transport of G-6-Pfrom cytosolo endoplasmic reticulum (and then Glc fromERo blood)(Glc)Chapter25Exercises1. (d) Leu -ketoisocaprate-酮苯(P645)(P649)(P647)酸(e)e enylpyruvate酸(f) Tyr AlaPyr;ydroxyenylpyruvate 对羟苯As

29、pOAA;Glu-KG2(a)AspGlc (by OAA)AAsp+-KG OAA+Glu(aminotransferase-PLP)B. OAA+GTP PEP+CO2+GDP (PEP carboxykinase-Biotin) (P573)C.PEP+ATP+NADH+H+2H2O Glc+NAD+2Pi+(enzymes flycolysis and F-1,6-BPase, G-6-Pase)(P571, P574)Asp+ -KG+GTP+ATP+2H2O+NADH+H+Total:+ GDP+NAD+2Pi1/2 Glc+ Glu+CO2+AspOAA (by Fum.)(b)

30、+A. CO2+NH4 +3ATP+Asp+2H2O Urea+ 2+2Pi+PPi+Fum(P636)B. Fum+H2O+ NAD+ OAA+NADH+ H+Asp+ CO2+NH4 +3ATP+4H2O+ NADTotal:+ NADH+ H+OAA+ Urea+2+4Pi+4. Pyr DH TPP转氨酶中 PLP（中的氮原子）以及Pyr DH 酶中 TPP 焦磷酸硫胺素（噻唑环中的氮原子）作为电子穴稳定负碳离子（中间物）(515)5. guanidinium 胍基（P635）N+ as electron sink6.底物中的标记氘转移到辅酶（与基交换）这个氘留在辅酶中，辅酶中另一氢原

31、子将产物中基换回基:具未配对电子,共价键均裂形成8. A. CO2+NH4+3ATP+Asp+2H2O Urea+ 2+2Pi+PPi+FumB. Fum+ H2O+ NAD+ OAA+ NADH+ H+C. OAA+Glu Asp+-KG+Total: CO2+NH4 +3ATP+ NAD + 3H2O+GluUrea+29. 2,4-二+2Pi+ PPi+NADH+ H+-KG (4P)肼（尿检） +质谱测定：deficiency of allthree enzymes complex(Pyr DH complex; -KG DHcomplex; branched-chain-keto D

32、H complex) (P645)the deficiency of the common enzyme “E3”(三个脱氢酶系共同含有的二氢硫辛酰胺脱氢酶: DihydrolipoylDH 缺陷)10.Cit瓜氨酸 +Asp argininosuccinate(byargininosuccinate synthetase 精氨酰琥珀酸酶)e 苯乙酸,Treatment: benzoate 苯甲酸,Arg, protein-restricted dietenylacetreatment: providing protein-restricted diet with large amounts

33、ofbenzoate (苯甲酸) andenylacee (苯乙酸) hippurate (马尿酸) (benzoyl CoA+ Gly) andenylacetyl glutamine (苯乙酰谷氨酰胺) (enylacetyl CoA+ Gln)排出：马尿酸，苯乙酰谷氨酰胺和瓜氨酸 (与治疗氨甲酰磷酸酶以及鸟氨酸转氨甲酰酶缺陷类似, P637)11. aspartame:苯丙二肽酯 （L-氨酰-L-苯丙氨酸甲酯；增甜剂）Chapter1. (A) Glycerol+ATPGlycerol-3-P+27通DHAP 还原生成(P606)(B) Glycerol-3-P + acetyl CoA

34、 lysoosatidate + CoAlysoosatidate + acetyl CoAosatidate 磷脂酸+ CoAosatidate +H2O DAG+ PiDAG+ acetyl CoA TAG+ CoA(P686)(C)FA+ CoA +ATP acetyl CoA+PPix3(P607)total:Glycerol+4ATP+4H2O+3FA TAG+3+7Pi+4H+(P1027)2.(A)Glycerol+ATPGlycerol-3-P+(P606)(B) Glycerol-3-P + acetyl CoA lysoosatidate + CoAlysoosatidat

35、e+acetylCoA osatidate磷 脂 酸 +CoA(P686)(C) FA+CoA+ATPacetylCoA+PPix2(P607)(D)osatidate+CTPCDP-DAG+PPi(P686)(E)CDP-DAG+Shosotidyl Ser+ CMPtotal Glycerol+ 3ATP+ 2H2O+ 2FA+ CTP+Shosatidylserine+CMP+2+6Pi+3H+(P1027)3.(a)CDP-DAG: activatedosatidyl group 活性磷脂酰( 从头合成)P687(b)CDP-enolamine（+DAG）: activatedosor

36、ylenolamine活性磷酸乙醇胺(节约利用途经)P688(c)acyl CoA: activated acyl group 鞘氨醇神经酰胺; 活性脂酰基(d)CDP-choline: activatedosoryl choline 神经酰胺鞘磷脂; 活性磷酸胆碱(P690 Fig 27-6 wrong pringwrong:osatidylcholine DAG; correct: CDP-cholineCMP)书中似有误(e)UDP-Glc(or -galactose): activated Glc group 神经酰胺脑苷脂;活性葡糖(或半乳糖)(f)UDP-Gal: activate

37、d Gal group (P690, 691) 神经节苷脂 GM2神经节苷脂 GM1; 活性半乳糖(g)GPPFPP; GPP 活性十碳(它的焦磷酸“尾”脱去)4.(a)MVAIPP(P693) 6C5C 羧基碳在脱羧时脱去(b)malonyl CoAacetoacetyl CoA (P693 and P617); 3+214C 脱羧时脱去both carboxyl C 羧基碳 losthe reactions5. No receptor receptors cant reach the plasma membrane （缺乏胞内转运信号或不正确折叠）fail to bind LDL norm

38、ally（LDL 结合功能域缺陷） fail to cluster in coated pits(不能在被膜小坑成簇；因羧基端区域缺陷).6.两个可能：DNA:（GA？）（CT）可能性不如 RNA 中（CU，仅脱氨）可能性大RNA ： C U (deamination 脱氨), so CAA(Gln) UAA(stop) and apo B-100 肝apo B-48 肠；（DNA）突变可能性不大（因肝中蛋白正常），是肠（转录时）RNA 改变（脱氨）所致7雄激素（androgen）的某些功能被二氢睾酮（dihydrotestosterone）介导，后者由睾酮（testosterone）还成，这

39、一步由依赖 NH的 5-还原酶催化。此还原酶缺陷的、为 XY 的出生时具内尿殖道（ernal urogenital tract），但（externalgenitalia）主要是女性的。他们通常被视为。到puberty），由于睾酮水平的升高而呈现阳性化（masculinize）。这些还原酶缺（testes）正常，而（prose gland）较小。陷的男子此信息如何用来设计一种治疗良性过度生长（ benignprosic hypertroy）正常老化（aging）过程中的常见病的药物？大多数 55 岁以上男子都患有某种程度的肿大（enlargement），这常导致阻塞（urinary obstru

40、ction）。Answer:testosterone睾酮dihydrotestosterone 二氢睾酮 (by 5-redue)theenlargement(肿大) can be treated by inhibitingthe 5-redue( 还原酶 ).Finasteride,the4-azasteroidogofdihydrotestosterone(二氢睾酮的-4 氮类似物), competitivelyinhibitsthe redue but does not act on androgen receptors 雄激素受体.Finasteride 药物还原酶 睾酮dihydro

41、testosterone(二氢睾酮)but testosterone 睾酮 normal prose()essmaller but testosterone-dependent proses (fertility 生殖, libido 性欲 and muscle strength) unaffected.8.drugidiosyncracies: 药物特异性;peoplemostsensitivetodebrisoquine（to treat hyperten）deficiency of a liver P450enzyme(ahydroxylasecatalyzing:debrisoquin

42、e hydroxydebrisoquine) gene deficiency: a member of CYP2 subfamilypayattention to exercising in giving other drugs to the above-mentionedpeople(the enzyme handles a broad range of substrates).9.deficiencyof21-hydroxylase,also11 -hydroxylase,17 -hydroxylase, 3-DH, and desmolase 碳链酶 adrenal hyplasia肾上

43、腺畸型生长virilization化 and enlargement of the adrenals肾上腺肿大。10.hydroobic odorants 疏水性有气味物质 are deactivated byhydroxylation.O2isactivatedbyacytochromeP450monooxygenase. NH as reductant.Chapter28(glycolysis)1. (1)GlcPyr(2)PyrAla(Glu; aminotransfer)total:+2.N2 NH4 (A)Glu (B)Ser (C)Gly (D) -aminolevulinate-

44、氨基酮戊酸(E)porobilinogen 胆色素原（F）Heme（G）+(A)N fixation, (B)NH4 assimilation, (C)aminotransf719), (D)FH4methylene, (E)与 succinyl CoA 缩合（限速步骤）(F) 二分子-氨基酮戊酸缩合 (G)血红素3(a) Gly+N5,N10-methelene-FH4 +H2OSer+ FH4 亚甲基(P719)(b)HomoCys 高半胱 + N5-methyl FH4 Met+FH4 甲基(P722)4（P717）5.isovaleric acidemia 异戊酸血症Leuisoval

45、eryl异戊酰CoA methylcrotonyl甲 基 巴 豆 酰 CoA(P645)（isovaleryl CoA DH）treatment: isovaleryl CoA + Gly isovalerylglycine 异戊酰甘氨酸（水溶性的, in contrast with isovaleryl acid）excreted bykidneys7.cysteine 半胱氨酸 and cystine 胱氨酸cytosol is more reductive -SH group within cells is not easilyoxidized由于胞内环境还原性较强, 胞内蛋白缺乏二硫键

46、,而胞外蛋白却含有(较多)二硫键8To and fro 往复，血红素的限速步骤（Gly+succinyl CoA）发生粒体（其它步骤均在细胞质中进行），原因是 succinylCoA 是柠檬酸循环中粒体内形成的9.dangerous trap的截获Pernicious anemia 恶性贫血(内因子缺乏所致 P644)VB12 deficiencythe methyl transfer reactionblocked (HomoCys + N5- methyl FH4 -Met + FH4)四氢叶酸供出甲基的主要途径被阻断 FH4 exist only in the form of N5-me

47、thyl FH4 “dangerous trap”(the reduction of N5,N10- methelene-FH4 to methyl FH4 is essentially irreversible, see P720).所以恶性贫血患者体内的四氢叶酸主要以甲基四氢叶酸形式存在. 其它形式的四氢叶酸(如甲酰四氢叶酸)缺乏,使碱基(Pu, PyDNA,RNA)得不到一碳的供应,迅速周转的红细胞无法.Chapter291.(a)Glc+ATPG-6-P+(glycolysis)(b)G-6-P+2N+H2OR-5-P+2NH+2H+CO2 (PPP)(c)R-5-P+ATPPRPP+

48、(PRPP synthetase)total:2.(a)Gln+2ATCO3 carbamoylosate+2+Pi+Glu（）(b) carbamoylosate +AspN-carbamoylaspar（）(c) N-carbamoylaspareDHO+H2O（）(d)DHO+NAD+Oroe+NADH+H+（）total:3.(a)PRPP+Gln5-osoriyl-1-amine (P741)(b) carbamoylosate +AspN-carbamoylaspar(P746)(c) Oroe+PRPPOMP+PPi (P746)(d)Nicotinate+PRPPNicotin

49、ate ribonucleotide (P755)(e)Anthranilate邻 氨 基 苯 甲 酸 +PRPP osoriyl-anthranilate 磷酸核糖邻氨基苯甲酸 (P725)4.Azaserine 重氮丝氨酸 as anog of Gln inhibits the step 1 andstep4inPursynthesis(P742),PRPPandformylglycinamideribonucleotide 甲酰甘氨酰胺核苷酸 accumulated5.(a)Ser+FH4Gly+ methylene-FH4+H2O (P719)(b)dUMP+ methylene- F

50、H4dTMP+ FH2 (P752)H+H+FH4+N+(c) FH2+ N(P753)total:The price of methylation: besides AA , reducinger required6.the synthesis of folate is inhibited by sulfanilamide 叶酸被对氨基苯磺酰胺抑制 (as anog of p-aminobenzoate 对氨基苯甲酸, one of the precursors of folate) 加入对氨基苯甲酸使此抑制逆转甲酰- FH4 缺乏inhibition of the step3 和 9he

51、Pursynthesis 甘氨酰胺核苷酸和 5-氨基咪唑 4-氨甲酰核苷酸积累细菌生长被抑制.7（a）Gln+PRPP 5-osoriyl-1-amine (P741) Pu Nt(de novo synthesis)嘌呤从头(b)hypoxanthine(Guanine)+ PRPP IMP(GMP)+PPi (P744)PuNt (salvage pathway)补救途径嘌呤核苷酸(c)Oroe+PRPP OMP+PPi(P746) PyNt(denovosynthesis)嘧啶核苷酸从头(d) Nicotinate+PRPPNicotinate ribonucleotide (P755)

52、 NAD+重要辅酶 NAD+的(e)ATP+PRPPosoriyl-ATPHis(：氨基酸)(f)Anthranilate 邻氨基苯甲酸+PRPPosoriylanthranilateTrp (P725)必需氨基酸的9. Dj vu幻觉 the step 7 and 8 in Pu Nt synthesis (AspFum,a NH2 provided) (P742);similarly:he urea cycle: Cit+AspArginisuccinateArg+Fum (P635);also, IMP(P743)10. Pernicious anemia 恶性贫血(内因子缺乏所致P64

53、4)VB12deficiencythe methyl transfer reaction blocked(HomoCys +N5- methyl FH4 -Met + FH4) 催化此反应的酶以VB12 为辅酶 FH4existonlyintheformofN5-methylFH4 “dangerous trap”(the reduction of N5,N10- methelene -FH4tomethyl FH4 is essentially irreversible, P720).No formyl-FH4left for synthesis of Pu Nt11.(a)ADA(aden

54、osine 腺苷 deaminase 脱氨酶)-deficiency (fordeaminationofadenosineor2-deoxyadenosineP756) accumulation of adenosine or2-deoxyadenosineS-A homoCyshydrolase 水解酶 activity ismarked diminishedlevel of S-AhomoCys elevatedS-腺苷高半胱氨酸腺苷+高半胱氨酸(b)elevated level of S-A homoCysthe activated methyl cycleblocked (P722):

55、 S-A Met S-A homoCys + CH3theactivated methyl cyclocked12. cell A lacks thymidine kinase 胸苷激酶 （for: thymidine todTMP）cell B lacks HGPRT (for: Hypoxanthine or Guanine+PRPPIMP or GMP +PPi) 两类细胞都是(节约利用途径核苷酸缺陷的)突变型i.HATmedium(H:Hypoxanthine次 黄 嘌 呤 ;A:aminopterin or methotrexate 氨基蝶呤或氨甲蝶呤; T:thymine 胸腺嘧啶

56、)Cell A(胸苷酸补救利用途径缺陷) : thymidine 胸(腺)苷dTMP (lacks thymidine kinase)dUMPdTMP (aminopterin or methotrexate)Cell B( 次黄嘌呤和鸟嘌呤补救利用途径缺陷): nosalvage pathway for Pu synthesis (lacks HGPRT)No de novo pathway for Pu synthesis (no materials)CellC( 缺陷互补 ):thymine(fromthemedium) thymidinedTMP(from cell B)PySynth

57、esis of IMP and GMP (HGPRT from cellA)Puii.cell A + plasmid (with the gene oferest and athymidine kinase gene)the only cellst will grow in a HAT medium are thoseve acquired a thymidine kinase gene.13. Adjunct therapy 辅助治疗:ancer drugcell killed NAdegradationPu degradationuratehyperuricemia 高尿酸血treate

58、d by allopurinol15. G-6-Pase deficiency(糖原增加)GlcATP(肝细胞胞质中) (降解)uratenormal people (by the ingestion of alcohol or by strenuousexercise) a lot of ATP consumedATP uratechapter30Liver contains G-6-Pase (so it can release Glc) and has no CoA transferase (so it can export the ketone body). Another diffe

59、rence: liver contains Glukokinase but no hexose kinase (thus only when there is high concentration of Glc does it take up Glc from blood)(a)GlcG-6-PDHAPglycerol-3-PTAGdeficiency of HK in adithe synthesis of TAGe tie woulderfere withglycogenG-6-PGlc (by G-6-Pase) export toblooddeficiency of G-6-Pase in liver glycogen in liver and blood Glcdeficiency of carnitine acyltransferaseimpairs theoxidationoflong-chainFA fastingandexerciseprecipie muscle crs(FA acyl-CoA acyl-carnitine transmembraneo Mitoacyl-carniti