ページ3:

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

ページ4:

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

ページ5:

.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

ページ6:

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

ページ7:

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

ページ8:

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

ページ9:

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

ページ14:

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

ページ15:

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

ページ16:

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

ページ19:

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
20017
Cuort
Coory
414
ALA
Succinyl-CoA
CH₂
sguthase
6A.34
'CH₂
Synthase
CH2
(4c)
2
CHOL
S-CA
--MH
+
"
alycine
(x)
{"
H
(6c)
S-Amandevulinate
(ALA) 0
COOH
I COOH
COOH
CHAH
CH₂
CH₂
SCH₂
21120
(COOM CH₂
CH
CH2 CH2
H-CH
ALA
dehydratase
WH₂
2XALA
WHZ PBG
heme (I) uroprophy rimogen
Cool
A[HOOL CM2 P
H₂C
31c
P
(first precursor pyrrole)
heme (II)=
Coprophyrinogen
20
Þ
4002
自発性
Type I
ANH₂
CH
Nˊ
H
Hz 4XPB4
uroporphyrinogen I
Synthase
Hydroxymethylbilane
(linear tetrapymoles
Zenzyme
uroporphyringen Te
Synthase
Type L
A
PA
I
Europorphyrinogen
P A
uroporphyrinogen
decarboxylase
PM
-1
Corprophyringen I
9002
PM
Proporphyrinogen Ty
M
Croporphyrhogen II
heme (IV) sheme
PPG
AP
A P
AP AP
synthase
ht
6H
61
PA
PA
AP PA
L
Proporphyrin
uroporphyringen uroporphyrinogen I
Uroporphyrin I
細胞質
IL
light
light
尿
wrdd
紫質→奋作用
64
64
(Corporphyrin Corporphyrinogen T
I
light
Coporphyriungen I Coprophyrin I
>
light
Loxidase
Protoporphyrinogen II L
oxidase
Protoporphyrinogen T
Ferrachel-Fe
ataje
Heme
Double A

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