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ページ1:
. 生物化學(二)表現part.胺基酸. Ch1. Brosynthesis of nonessential amino acid- →胎壁酸代謝概况. >胺基酸的簡介: →非必须胺基酸之生合成 1. Transamination As •21 Deamination area cycle 尿素循環 來源 組織 Diet pritein 修復 tissue protein Arano Acid 非蛋白質讓物質 ansamination Carbohydrate At glutamate Acetyl-CoA ketone bodies ↓ Lipid: (TCA. gle Aurea 箭頭的方向 C02 .肟基酸基本結構 鹼性 H2N-C-COOH 酸性 腰茎 R 羧基 側鏈 重要的: AB (Ala) R7-CH₂ (3c) ·天門冬醯胺(Asm)R=>-CH2-CONH2(4C)中性脂基酸 天門冬胺酸(ASP)R=)-CH2-COOH (4K) 酸性 A (Glu) R= -CH2-CH2- Goon (5c) 324 .麩胺酸醯胺(Gln)R=>-CH2-CH-CONH2(5C)中性 非必需Amino Acid Alanine Arginine Asparaginė Aspartate Cysteine. Glutamate Glutaminer Glycine Proline Serine Tyrosine. 必需Amino Aid Histidine. Isoleucine Leucine Lysine. Methionine. Phonylalanine Threenine Tryptophan. Valine. Double A
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Transamination NH> -OOC-C-R H amino acid HUDC-C x-ketoacid -CH₂CH₂COO". x-ketoglutarate ($C) Deamination WHY -OOC-C-CH₂CHICO- WH -00C-C-CH₂CH₂ COO- α-KG Fo Glu₤101 glutamic acid 1994 Gluca. (50) → 647-1954 લ 4 Glu 'H' Glutamate in liver. H 0 + Glutamate dehydrogenase Aα-KG A 2132 other thing 2-ketoglutarate Glutamate 453 (Glu) =0 ·α-ketoglutarate aa H 11 NH3+ L-Glutamate H20 NAD(P) NAD(P)H +HT 需耗能 2. Glutamine: 644 65. (Gth). NH L-Glutamate L-Glutamine WHI Mg-AIP Mg-ADP- 耗能. 1. 1. 12 glutamate delydrogenase 211887 as 547-35% 不反应可逆 4. WHAT BENADI. ⇒多餘脂换掉 ' 1. glutamine synthetase " ,與Glu合成均需能量,此為Mg-ATP 3. WHAT HE 血液中游離腰 →血腰濃度过高,肝無法完全處理 放先將脢存在Gln上. 7744131 Double A
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3. Alanine and Asparate. 21 pyruvate (30) OAA (4) WH. Gulor Asp JAIR WHY "Alanine (30) Asp (40). fa-ketoglutarate for 1973773 GOT: GPT 64% glutamate-pyruvate aminotransferase Ryalanine transaminase (ALT) (GPT) for. oxaloacetate OAA- Asp 533 = GOT ROY AST 血f GPTM,GOT---(膜破,核後破). (5) denature 液 GPT IT GOT IT (A) # he cross細胞死亡 1973546) GPTTT, GOT-- (ATTE 9776(37%) if all 362.1224 54 GPTTTT, GOTT 4. Asparanine 255 →游離胺过多,GM主变GM仍不夠時, 則用此 L-Aspartate 30: 4C: &C: . [Ma-ATP .耗能 . L-Asparagine Glu Mg-Aup+ppi α-keto acid NHS. Amino. and pyr ANH Al OAA. > 第二道解毒系統(血脂高時) > 需耗能 G → Asn synthetase Asp IN Ash. A α-KG NH Glu Gl DAA+NA B-NH3. Glu A→ Asp B7Gln Ala-HA Glu+NH₂ B ·A → Pyr B7 Gln. Double A
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8. Cysteine 2.4.
H₂D H₂N
Met +Ser
SH
Homocystein
+
HO.
"
H.
TH₂N
Coo
Serine
1.主要骨架得自Ser
H₂O
-000
HH,
THIN COO
Cystathionine
21S由必需胺基酸(Met)合成而来.
COD
α-ketobutyrate Cystein
.半胱胺酸
因此甲硫胺酸為升糖胺基酸
Cysteine : Serine({{1) & Methionine (19)
9. Tyrosine 27 155X
Phe hydroxylase complex
L-Phenylalanine
→L-Tyrosine
H2O
一个口接上,一个口变水
提供行搞上
Tetrahydrobiopterin 16
10, Hydroxy - proline- and Hydroxyl-. lisine,
Phe hydroxylase complex.
→ifika
→ ZE SUPKU BRIGA GUE
α-ketoglutarate
[180] Succinate
A collagen
1802
Ascorabate
Vitc
protein
Promm Prom Lys Glyn
"RNA · Pro/Lys Hydroxylate
↑.
DNA
1½ 1, Ep 1½ ½ orj
• Vicca 2. Collagen S. Scurry
11. Tyrosine and phenylalanine.
Coo
HO H
Choristmate
ASA
"C02" 600
605
04
- glutamate α-KG
phenylalanine
Ho H
y
Prephenate. NAD
NADH
Phenylpyruvate
Glu α-kG
002
OH
OH
Tyrosine
12. Tryptophan 2 ½ 1354 in 12 7245
Choristimate
→Anthranilate
n
Gin. Gluthyruvate
.
G-3-P
提供口
1.N-anthranilate.
PRPP PP:
Ser→ Try & Gily
Phe hydroxylase 16
→此酶只存在哺乳動的肝
Indole Trptophan.
1₂2₂0
Double A
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.5, Serine 2451 OH Yo NADH. 0 Ro 0-0 。 生合式的来源 → Glycose D-3-Phosphoglycerate Phosphohydrony pyruvate (6-3-0) α-AA 2-24 1344 2-KA 2. 什么AA都可以 HO L-serine (30) 2-0 0 Phospho-L-serine 6. Glycine 27 E51 TATR 3, 537. CH₂ CH₂ 酵 Choline. -CH3 CH3 24 CH₂ H3C-N-CH3 0 Betaine aldehyde Dimethylglycine CH₂O CHLOHA I NADT H₁₂C-N-CH3 Betaine HO 帶走單氮+官能基 WH3 Hy folate Serine (30). Methylene Hy folate. NH ☆☆第二種方法:From serine Cilycine (2cj serine hydroxymethyltransferase Tertraphydrofolate (Hy folate, → 2 Hy folate Glu +NH₂. 0-14 Ala Glyoxylate Pyr. Glycine. Sarcosine 第一種方法: CHT Glycine From choline Choline Glycine 3CH₂ -7. protine 23 (5c) 6 NADH. 还原 H2O L-Glutamate kt. *=72592 = From Glyoxylate 7. NH3 Glu or Ala #6 13. 妣 -Y-semialdehyde 还原 a. 半羧醛 L-Glutamate-r. -NH₂ L-Proline WADH s²-Pyrrolidine-5-carboxylate 或環 Double A
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protein and amino acid nitrogen Ch2. Catabolism of →胺基酸代謝-總覽. →氣的代謝與urea cycle 胺基酸代謝. Dietary protein Body protein. 75-80% Liver Amino Acid. 代謝. 'sythesis pool nrtiogen compounds. citric acid cycle urea cycle 51、DNA RNA鹼基 •(WHY") .21 Heme &貝類似結構 .31.神經神導物質 4.荷綠,磷脂質 •Dietary protein digestion protein proteolytic enzymes Amino Acid] Body protein turnover 1 +ATP VPi WH 自用 E3 `AMP →到腸→到血液 AMP E2: ubiquitin (321) 18X2 KE E2: ubiquitin共轭酶 [3: ubiquitin 泛素化 胜肽片斷 ↓ protein 197 Amino Acid. \- urea cycle -> 碳肯架. &肝 ↓ Kett 脂肪酸合成 呼吸 Double A
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3. Ammonia transport Glu →Glu Miglutamine. synthase Flex. Ala (Asins, and) → 573-434 6 16 2 (glucose-alanine cycle). → Alanine 19216 50% Glucose GA7P A Pyruvate Urea ATP 14H₂ NHz 2-KG at Glu Ala. Alanine liver 4. Urea cycle (in liver) CO₂ Acetyl-CoA+Glu Activator. Burea cycle开始 ZM-ATP -Acetyl- glutamate >Mg-ADP +Pi ic 5748 0 -c-10-p-0 Carbamoyl Phosphate 粒 A7 Glucose ZATP (Pyruvate (2) Lactate -amino acid ALF Ca-beno acid Blood Alanine Muscle Carbamoyl phosphate (WHO) sythase I CH₂NH₂ 558 H CH · CH₂ Coo (L-Ornithine omithine Ure transcarbamoylase 反应过程 Ne CHÍNH CH2 CH2 Coo D bc L-citrulline 果胺酸 (Tu!) NH [OH] WH WH₂ CH₂ · arginase 60 Coo L-Arginine 裂解@ argininsuccinase argininosuccinic acid sythase Coo Mg-AIP AMG + Mg-ppi HC-COOTCA. -00C-CH Fumarate NH COO. CCH CH₂-NM L .CH2. M₂ Coo Argininosuccinate CH₂ H-C-N CH₂ L-Aspartate (4c) 3.兩個氣(H)來自哪个aa? →Glu >Asp 1.721. 2、位置 enzyme=54 Co₂ = 1 786741 →細胞質內 WHY: I ATP (P;): 3 (4) H20=1 Double A
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ch2. Urea cycle → A3 #=218618 in Body.. →不會當熱量來源,直到脂質,糖類耗盡 84 & 7+714 Aurea cycle Urea #135 1 1. Transamination 14. 21 Oxidative deamination RIGAAT 3. Ammonia Transport Abi | Fj 4. Reactions of urea cycle (in liver) → 1 2 3 KI-4 musele ka-aa- D→Ala My & Pyr liver 13124. α: AA. L-KA (Ala) of ketoglutamarate L-Glutamine, ②氧化 肷胺 Suren cycle Urea. 41a120%70-0 Glu(2)(20%) liver Aila 100 Gly (10%) Sr. other Val 直接到肝. Pyruvate α- Amino and Ala aminotransferase ·L-Alanine > ketoacid. 毋受考 a-ketoglutarate - Amino acid Pyridoxal phosphate (PLP) Glu aminotransferase L-Glutamatek Other: lysine, threonine, proline hydroproline -keto and. 19 Ala 979 30% +20% = 50% Ala aminotransferase = 317 3=15 1 AT Glu Glu aminotransferase 14- 2.氧化脫腰 (need Glu dehydrogenase) •L-Glu→ L-ketogutarate → in liver NADIP L-Glutamate 个 all-AA NAD(P)H+H. •L-Gln L-Glu (need glutaminase) TH₂N (NH) →urea cycle H₂O x-ketoglutarate 去途 6 " L-Glutamine °-2/ Glutaminase 抗氧化酵果3个 → catalase: SoD, GSHP →α-keto acid. •α-amino acid 2. (need amino acid oxidase) → liver and kidney RH'C" •ox-Amino acid amino acid oxidase Flavin. Ravint 75627460202 catalase 1×0₂ 裂解 20 irea MHJ R-GC-O- x-Imino acid H₂O₂ WH cycle x-koaúd
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尿素循環代謝異常缺乏酵素與症狀. Hyperammonemia Type I Carbamoyl phosphate 新生兒方面氤,意識模糊;呼吸急促,発育遲緩.. (CPS 1 Deficiency? Synthesis I Hyperammonemia Type I Ornithine Transcarbamoylase (oTc Deficiency)比例最為 →高血胺症,危險 新生兒高血氨,嗜睡,嘔吐、癫痫、昏迷 Citrullinemia ·果胺酸多. ·Arginio succinate .sythetase. .高血氨,虽愁不振,弊再遲緩,肝功能異常 ·Argininosuccinic. ·Aciduria 堆積 Argininemia Arginosuccinicase .高血氧,肝腫大、脆性毛髮,或發生長,認知障礙 (Arginiosuccinate (yase). →積極治療可活: Arginase 漸進性癫痫,運動功能障礙,僵硬,智力低下 不會死. 臨床表現 →篇花新生兒期兒童期笄病 ·急性高血氣症:意識混亂,呕吐、癫痫、昏迷 神經系統症狀 ·肌肉與運動異常 ·其他:厌食,肝功能異常,呼吸急促 →嚴重的高血氣症可能導致腦水腫、神經損傷,甚至死亡 治療策略… > 急性期治療:降低血氬. → 靜脈注射阿洛魯酸鈉&丁酸鈉 →透析(血液透析or腹膜透析). 補充必需胺基酸 →精胺酸(Arginine). 1 瓜腰酸(CGtrulline? 高度與高脂肪供能 →避免蛋白質分解、減少氣產生. →長期整理:低蛋白飲食(発育易不好) 特殊配方奶粉與胺基酸補充 ˙苯丁酸鈉or苯乙酸鈉持續治療 基因治療&肝臟移植(根本治療) Double A
ページ10:
.ch3.. 胺基酸的碳骨架代下: → WHY Urea cycle 11 Carbohydrate & Fat → 胺基酸代謝異常的疾病,may造成腦死亡or傷害. 治療:減少攝取代謝有異常之AA: 血液運朽將正常酵來取代異常之酵素 Gene 生糖與生酮性胺基酸 7. pyruvate or ·TCA cycle :P) 472 aa... 124 = glycogen. ·Serilys.. Met Val 11. Arg... Asp: Glu, Ash Gln, His: Pro, Gily. Ata. 14: Acetoacetate, acetyl-CoAzaa, LX55X fat ** →生糖與生酮性: Phe Tyr. Trp., Jile, Lys. Thr 芳香族 77 胺基酸代謝與中央代謝途徑之關係. Ala [Arg. Cys Glu fa-ketoglutarate ← Glutamine- His Hyp ser Le Zeu Thr Citrate Trp Succinyl-CoA Pyruvate Citrate. . Gln -Pro- :Ile Met Val Acetyl-CoA cycle ↑ Acetoacetyl oxaloacetate Fumarate Tyr -GA Lenys Aspartate & Ash Phe. Trp. Tyr li Asparagine and Asparate· Oxalacetate T Aspartate Asn4c ·α-ka. Ga H₂O C WHY NH2 CH2 Ala liver Glu urea cycle 1700 CH2 H-C-NH₂ Asparaginase 1. 405 L-Asparagine CH2 H-C-H₂ transaminase 'coo L-Aspartate Oxaloacetate (OAA) Double A
ページ11:
•Ornithine Transcarbamylase 1 1 $267 50% • Argininosuccinate Sytherase (Ass) * 2/2 2:147 • Arginase (ARG) TETEP Carbamoyl Phosphate Synthetase 1 (CPS1) - 1 @Citrullinemia the Taiwan th31ts of RK (Arginino succinate synthetase) 遺傳性的高血氧症 SAXH Glutt, Citrulline↓ → • Dor OJK 2, PRS Month - 2 1. Sodium benzoate (Ucephan) = . Gly 10%. (glycine (hippurate) 785 Urea Cycle. ·2. Sodium phenylbutyrate (Buphenyl)=. 16 phenylacetate, glutamine, phenylacetyl glutamine to the f 3. Arginine * arginase (287) arginine 145 (Ornithine) 氢毒性腦病 KX.氨濃度↑ TCA. 2kg.x. Gl 會穿越腦中屏障(Brain barrier) if #56 1, Glucose 537A, α-KG 5379. R • TCA cycle EP73. ATP 735-91158.138 ZTQ 354 Glu 67 33347 GABA → (476||12) WHITTCAX, ATPX, GABAT .蛋白質減肥法 →用上述机轉,但在体cell Glex acetyl-CoA TCA < 77.62 week. →只吃蛋白質,不吃醣類. Glu ürea cycle Double A
ページ12:
Glutamine and Glutamate. of-ketoglutarate & Glutamine czo ka dina WHO WHY TArg Hiz ain sc -Pro sc C=0. Ala CH₂ 6112 CH₂ CH2 CHL glutaminase H-C- transaminase coo ' 1807 500" L-Glutamine L-Glutamate .α-ketoglutarate 3. Proline proline L-proline NAD? dehydrogenase; NADH-TH AR H₂d XH+ • Hyperpolinemia Type 1 proline dehydrogenase →常抓狂,智障种问题,檢椭発作 ● Hyperpolinemia type II = →狂较少,比Type工輕微,痙攣,抽搐. L-Glutamate-r-semialdehyde, Glutamate h dehydrogenase NADT Semialdehyde NADH+H glutamate L- K-ketoglutarate 41 Arginine CH₂ WHIT 醛基 WA H₂O L-Arginine 24 Arginase 4H WHIT CH₂ ·CH₂ L-Ornithine 2-19 2 Glu CH L-Glutamate-r-s " r-semialdehyde Arginase Double A
ページ13:
5. Histidine. H₂O L-Histidine Histidase HN JH. urocanate . Varocanase if Histidase 23372 Figl474 >缺乏症狀:过动、語言障礙,発育有问题,學習困難.. 4-Imidazolone-5-propionate imidazolone propionate hydrolase <H2 CH A 檢測:給大量Hs " if Figu N- Formiminoglutamate (Figlu)) 72 TH4. Folate] glutamate. Formimino. Transferase. ·NE Forminino" Hy Folate L-Glutamate 2-ketoglutarate Cys Gly1(30) Hyp Ser (30) Jhr. (a cystine 胱胺酸→很難溶解,可能會在胱胺酸尿症病人腎小管中沉澱,形成結石 NH3+ H₂C S 30. 30. Cystein - Pyruvate (2ways) Cystein NHA 4. L-cystine cysteine HS ters (bc) dioxygense WHIT Pyruvate S Acetyl-CoA Citrate MM •NADH+H! Cystein. Salfinate 'transaminase ② Cysteine transaminase -AA H₂C 0 METS. ·HS. -keto acid •o-Amino acid · Pyruvate 再动脚 Cystine reductase NAD 2X NH3 sulfinylpyruvate L-Cysteine (c) *ALAGER desulfinase soy Pyruvate Hic 先动胺 ・0-3-Mercapto pyruvate (thiolpyruvate> H₂C NADH+HT NADT OH " 3-Mercaptolactate Double A
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2, Hydroxy-proline H H 3. Threonine (40) NH3 H3C CH CH T 011 " 。 L-Threonine 4-Hydroxy-proline 24 t NHT •@ Hydroxyproline dehydrogenase ifakib 跟以前的一樣 。 L-O'-Pyrroline-3-hydroxy-5-carboxylate 04 threonine aldolase H3C-CH " 0 Acetaldehyde NAD+ aldelyte Kimomunit H₂O. nonenzymatic dehydrogenase. " 0 -Hydroxy-L-glutamine -- semíaldehyde H₂O NAD NADH+H To dehydrogenase. OH Erythro-r-hydroxy- L-glutamate → LEG»ICA transaminase 2-AA Acetate CASH / Mg-ATP H₂O! Mg-ADP 升糖 Acetyl-CoA Acetyl-CoAt Fumaratek Tyr. Ile. Len Phe Acetoacetyl-CoA Trp Pyruvate Len, Lys.. Succinyl-CoA Met Phe, Trp [Acetyl-CoA] Tyv: 2 " α-keto-r-hydroxyglutamate Glyoxylate 乙醛酸 Galy - 1 Phe and Tyr → Acetoacetyl CoA and Fumarate th Trp Acetoacetyl CoA and Pyruvate. 3. Val Propionyl CoA.#. 21 an aldolase Pyruvate 4. Ile Propionyl CoA. and Acetyl CoA). 5. Len-Acetoacetate and Acetyl CoA A Acetoacetyl CoA acetate, acetyl-CoA. >>> Double A
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苯胺基丙 phenylalanine ②.代謝 苯丙基丙酸 tyrosine 酸羥化酶酪胺酸. 往这走 1. Phel D CHANCH-2007 WHY L-Phenylalanine -ketoglutarate α-KG L-Glutamate Glu. 内 轉腰 酮酸 1. Phenylpyruvate 代謝異常產 NADT ·NADH TH+ H20 Phenylacetate L-Glutamine ·H2O Coo ·NADHHHT →NAD+ 3" Phenyllactate ①if缺乏 Phenylketourea (PKU)= 苯丙酮尿症: →体染色体隐性遗体 →Phe(苯丙腩酸)羥化(hyhongation) 或Tyr (酪肟酸) 代謝途徑異常所致 →目前已知5種酵素異常会導致此病: 苯丙胺酸羥化酵素 phenylalanine byhorylase; PAH) GTPCH, PEPS, DHPR, PCD. →症狀:精神病,濕疹:鼠臭味 → 智能遲緩,IQ270. →以低phenylalanine飲,直到6歲 → 阿斯巴甜 Asp與Phe結合,甜度200x CM2 C+12 CONH₂ phenylacetylglutamine ③ cause Pku 1.2.3→管會從尿液中排出 2. Tyrl. →Tyrosine melanin phenylalanine 黑色素 画画 尿黑酸 DOPA ↓ homogentístic and catecholamines acetyl CoA fumate Albinism = 516. →動物較易罹患 → Tyrosinase (6 $11663}\ →if部分缺乏黑色素,稱局部白化病 →易皮膚癌、眼、皮膚異常敏感,虹膜呈紅色 兒茶酚胺 乙醯COA 延胡索酸鹽 Double A
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CH₂ Tyrosin OH L-Tyrosine homogentisate. oxidase H20 Glu CH Transaminase OH 0. Ascorbate co P-Hydroxyphenylpyruvate hydroxylase F¾A P-Hydroxyphenylpyruvate" dka " 0 Maley lacetoacetate CH 11 (rewritten) Maleiylacetoacetate C生糖性 0 尿黑酸 Homogentisate Glutathione maley lace toacetate is. tans isomerase F@BR-Hydmyphenylpyruvate 大量堆積→中毒,嬰兒血症. Fumarylacetoacetate fulmary lacetoacetate hydrolase Fumarate H₂C CH₂ " Acetoacetate COASH ˙g-kerothiolase J&c. 5~46A 0 「Acety-CoA) 「Acetate Neonatal tyrosinemia (発兒酪胺酸血症) → Hydroxyphenylpyruvate hydroxylase →寶寶有生煮捲心菜味 →f來治療,肝、腎衰竭 Tyrosine phenylalanine #1 → TU¾ biz & =L·K (@Kyr). 3.. Trp.1 →植物的皮多有. →打斷钚加氧 生酮 Alkaptonuria Aude →無味尿黑色 →缺乏 Homogentisate oxidase. 尿黑酸堆在体内並排到尿裡,空氣中的 氧化三,使变黑色 →40吖後,因尿酸被polyphenyl oxidase氧化.. 使末期発生祃菜病,关節炎. →堆積在耳朵軟骨,耳朵变器... →堆在眼睛澤膜向膜出现黑斑 →关節発炎、結晶: 14/2 HP pyruvate (TRAlaF), acetyl-CoA., acetoacetyl-CoA Double A
ページ17:
4. Val. He, Len H3C CH3 WH L-Leucine (bc) " H3C-CH-CH-C-O " 。 L-Valine (sc) 13AVIA | α-ka 50, 40 50 CH₂ H3C- \CH- CH "1 0 L-Isoleucine (bc) acetyl CoA acetoacetate propionyl CoA 51712 acetyl CoA B propionyl CoA 472. if缺陷x-同基酸脫氫酶 Maple syrup urine disease (2; MSUDD → Brunched-chain keto acid dehydrogenase BASLAíàng →酮酸在尿液&血液堆積 →尿中有枫糖蜜味而得名。 → 易発生於原住民 →腦病变、酸帡 →終身的營養治療 1717 3K 25536771554 thiamine pyrophosphate 5. Met] [Succinyl-GAJ = [" NH3 Ile Met val H&C CH₂ -0- NH3 " Cystathionine L-Methionine ATP S-Adenosyl-methionine Acceptor H2O 'CH' SH WHI CH₂-Acceptor L-Cysteine CASH. S-Adenosyl-L-homocysteine [α-ketobutyrate NADT NADH+H+ Cox H₂O Adenosine NHSt H-SCHCH₂ + L-Homocysteine cystathionine Glycocyamine 0 Creatine phosphate WHE L-Serine ß-synthase H₂O H₂G CH₂ " S~GA Propionyl-CoA ↓ TCA cycle Met = 144 Ha-folate=4 -Cell膜上→竿碳儲存 Double A
ページ18:
Ch4.胺基酸&其產物. ☑ - → Glycine 11/9357: Heme. purine, creatine T 1 9 Alanine Gly Arginine and Ornithine : FRA Urea cycle. spermine等多胺類之前要区物,多脾類之 Ornithine 管理作用為降低体温及降血,Arginine 會產生No幫助血管擴張 Tyrosine Dopamine, AR± Tryptophan: il Serotonin (142), Melatonin (BBE7A9 765) LV Glutamine: 555 Pr-Aminobutic acid (GABA), 14707 J J Z R & R z JP 3 ) 1 4 F £ £ ¥ ¥ 1. Glycine 6445544401 H₂N: C NH₂ TH₂O Vrea. ' CH₂ CH₂ (kidney) Arginine-glycine transamidinase WH₂ L-Arginine HẠCH CH *H₂N-CH₂-COD Glycine [Ornithine Glycocyamine & ✓ acceptor (guanidoacetate) (Liver S-Adenosyl methionine ATP ADP Guanidoa cetate methyltransferase S-Adenosyl methionine WH nonenzymatic in muscle HN=C HN=C \N-CH₂ urine Pi+H20 Creatinine 腎功能指標 2. Ornithine 933447. proteing 1L proline orthine … Glutamate-r- semialdehyde ↓ Glutamate urea 'N-CH2-COL CH3 creatine phosphate < CH₂- acceptor protein nitric oxide No Arginine creatine phosphate, creatine arginine phosphate putrescine spermidine spermine WHE H3C Methionine Spermine growth factor: cell 315 Pharmacology: 44.145 zw Hz "WH₂+ Spermidine -0- Decarboxylated! S-adenosylmethionine spermine CH Methylthio- A adenosine synthase H Double A Spermine
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3. Tyrosine 5333544 牛肉中多 Ho. 動物性蛋白 CH₂ 含量多. Tyrosine L-Tyrosine Hydroxylase k HO Dopa Dopa • decarbonylase CH PLP decarboxylase Co HO- CH₂ CH₂ Dopamine Dopamine Ho B-oxidase Cu 04 HO. " \NH tamin C CH-CH₂ -NH₂+ phenylethanol-S-Adenosylmethionine A amino Nu-methel transferase-Adenosylmocysteine Norepinephrine <} *A Ho. OH 07 -CH₂ M Epinephrine A 腎上腺某过多:心都不事 过少:动作过慢 ICH Parkinson's disease) → 15 Dopa 59573445 ·L'dihydroxyphenylalanine 加上 monoamino oxidase inhibitor. →腦部黑質&藍斑内合成 Dopamine by cell →通常老化不会造成要再加上 环境因子(若器制) →7372=677 H3C 4. Tryptophan 54433447 514767534 Ho NH₂ ·OH L-tryptophan (10 tangtophan Chydroxylase NH₂ OH 5-hydroxy-tryptophan HN aromatic amino and decarboxylase NH2 serotonin 5-hydroxy-Tryptomine (5+17) N-acetyl 血清素 transferase Ho HN 。 N-acetyl-5-47 15-hydroxyindole- o-methyltransferase HN- -CH3 (melatonin) 褪黑激素 Serotonin血清素:陽光促進 調節体温、心情、睡眠、性慾,食慾 →足夠即往好的方向進行,不足则反之 →使Tryptophan成為抗憂小藥 → 香蕉皮15t(綠),奇異果(all) 2nd, A receptors (transporters Melatonin褪黑激素,陽光抑制 調節生理時鐘,強抗氧化前(保護粒綠体DNA,細胞核→ 76753 Double A
ページ20:
Glutamate的衍生物 L-Glutamate decarboxylay 100 HC-NH, ' CH₂ CH2 coo (5c) L-Glutamate 102 = H3N-CH2=CH2-CH2-C00 -Aminobutyrate (4c) 丁酸(GABA) ↓抑制 中枢神經系統 市面上常見助眠 → GABA+Melatonin 芝蔴明e→沒用. 麩酸內反應 味素(精) →有些人对商量麩酸鈉異 「中國餐館症候群」 (頭痛、冒汗,反胃灼熱、刺痛for 30min) →GABA太多,抑制神經系統的保導 不傷害 @va 結晶 鮮味來源 →限鈉飲食患者不能吃 可能会引発人板氣管痙攣氣喘, 也是一下子而已 Double A
ページ21:
Ch's Porphyrins and bile pigments 紫質、血基質前身。 heme575 heme的代謝 →肝臟的構造 →血色素合成 →膽紅素代謝 →膽汁酸代謝 一胰臟疾病的分類→黄疸 肝臟 liver cell #距維相等→快 >人体最大器官 →75%流向liver下血液,源直腸腫的靜脈 →昕構成膽汁要素為膽囊&膽醬 7 血色素的合成 →血紅蛋白,肌紅蛋白,細胞色素的組成物 € + porphyrin + Fest → Porphyrins $34ĭ pyrrole rings IX CHF $51 + Fe Pyrrole ring 2 NT48 binding Uroporphyrin TL (1) Alacetate)= CH2000H BC propionate) = CH₂CH, COOL) agv無照顺序. → Uroporphyrin 亚 Uroporphyrin and Corproporphyrin(粪紫質) A. P AP HC CH HC ~~ H 111 CH Pyrrole t吡咯环 HỌC LỊCH TINY HN HC- CH porphin (C20H144) PA Vroporphyrin I P M PA UroporphyrinⅢ MP PM Corpropurphyrin I Corporporphyrin IL M(methyl)=CH3(甲基) > 最早在urine(fi発現 >11feces().. Double A
ページ22:
25 of heme (2) porphobilinogen
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Coory
414
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I COOH
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Cool
A[HOOL CM2 P
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Coprophyrinogen
20
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自発性
Type I
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CH
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uroporphyrinogen I
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Hydroxymethylbilane
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PM
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ページ23:
8 Fe² Heme vint Protoporphyrin n 1/ Photo 6 胞体紫質況者病(The porphyrias) →血色素造成上有缺陷 DNA突变 gen IL) Photosensitivity ALA, PBG Coprogen =5~8 堆積 1.ACA阻礙神經 性 聲望你堆程 ↓ Lysosome ↓ $4th enzyme 組織的ATPase 5 × Uro TV 2、腹部吸收过多 ALA -gen 皮腐傷害 ↓ Hydneymethylbilane • T ↓ 腹痛神經精神病 photosensitivity #hitek PB. ALA, PBG-13:13.4. ALA Ala sythase Succinyl-CoA 為Heme关建酵素 Glycine Enzyme 缺乏&症狀 ①ALA sgthase → Anemia貧血 ALA dehydratase → Abdominal pain ③ Uroporphyrinogen I synthase→肚子痛 肚子痛 ④ Uoporphyrinogen Ⅲ synthase → 不會光过敏 Uroporphyrinogen decarboxylate → Photosensπinty th ⑥ Coproporphyrinogen oxidase →光过敏,肚子痛 Protoporphyrinogen oxidase tak ⑧ Fermochelatase →光过敏 → 急性紫質症未接受適當治療→嚴重周邊神經病變 Double A
ページ24:
膽紅果代謝 →在肝cell代謝,膽汁系統排出腸 120天 → If 代謝異常,膽紅果會分送業色色来至皮膚 正常代刊 黃疸,尿颜色深 N → Fest 各種中間產物的颜色?&水溶性? cooly heme heme OLINADPH Fetty oxygenase NADPT triso bliverdin膽綠案」→水溶性,綠 MOOC Cool! unconjugated bilirubin 1. 未結合(未溶於水) (uptake) 與albumin (白蛋白) ↓ 與葡萄糖醛酸結合(conjugation) 肝 (增加水溶性) 結合 AALA 膽紅菜 →不溶於水,荧 bilirubin 腎 尿膽素 尿液 醛酸 (部) 美腿系 + feces粪便 Conjugation. Gibcode UDP-Glucose dehydrogenase UDP-Glucuronic acid. delyingmase ZNAD 2NADH+ZHT UDP-Glucuronic acid VDP-Glucuronic acid transferase + Bilirubin VDP-Glucuronic acid 同上 Bilirubin monoglucunhide 水溶性不夠,30再一次 Bilirubin diglucuronide (12) Bilirubin manoglucuronide (UDP) Bilirubin diglucuronide -000 (CH20) 4C-0-c " c H₂C CH₂ 。 M V H² CH2 M -(Secretion) 分泌 Double A
ページ25:
肝臟疾病 → 肝炎:A、B、C型病毒、酒精、乙醯氨基分. 発炎6Month↑→慢性發炎 →膽汁閉塞:肝臟丰身膽汁專營小,外膽汁導管校大 →黃疸(jaundice):膽紅素菜常,分肝前,肝中肝後 Acetaminophen 與肝臟衰弱 普拿糕. 血 正常:與尿肝毆or硫結合,由kidney排出. 膽 过程:被liver cyt p450氧化 ↓ N-acetyl benzoquinoneimine Blood ↓ 細胞过氧化 Bilirubin -Albumin 1. uptake (ATF)) ↓ 肝損傷(急性) HEPATOCYTE Bilirubin 補救:結大是Glu 21 Conjugation (A+) Bilirubin diglucuronide 酒精有关的肝疾病、 TSB →纖維化→肝硬化 肝癌 BILE DUCTULE 3. Secretion(肝後) liver (芝能出现) Bilirubin diglucuronide 肝前→活血(危險,紅血球破下多,膽紅素竹→共疤) 肝中→感染(A、B、C型肝炎) 代學品/藥物(止痛制工醯氨基酸,酒精) 遠傳性疾病(Gilberta,Crigler-Nejjar, Dubin-Johnson, Rotor症候群) Bilirubin UDP-glucuronyl transferase. 遗传性 自体免疫 新生兒(生理性) 肝後→肝內膽管(藥物、慢性阻塞、膽管炎 肝外間管(膽結石,A腫瘤、肺症) Double A
ページ26:
黄疸文化权值. Normal:結合: 0.1-0.4 : 未結合: 0.2-0.7 末结合个 肝中:直接&同接↑他得看AST、ALT.GABA) (酵素異常、肝本身有问题) 肝後:直接个 肝前共疸(溶血,大出血) @ 未結合型↑ 皮腐芝 F= bilirubin t 肝 尿液色 肝內黄疸 血 AST 血清↑ 肝 B 尿液个 已結合 粪便止 尿来 肝後芠疤 芝 肝 尿菜 美浅色(完全下不来) 初生芠业(50%) transferase (UDP...-- ) →三天発展共担 →生理上,膽紅來結合暂時無效,10天後消除 →很嚴重,需光線(紫外線治療→變化 更→輸血,以避免後其在造成腦傷害 →一出生有共担不正常,10天後才出现也不正常. Double A
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