CHNG XIV

CHUYN HA PROTID
MC TIU
1. Phn bit c v k tn c cc acid amin cn thit v acid amin khng cn thit.
2. Trnh by c cc bc ca phn ng chuyn amin v kh amin.
3. So snh c sn phm thoi ha cui cng ca ba nhm amin, carboxyl v
hydrocarbon.
4. V c s tng qut ca cc bc trong chu trnh tng hp ur ti gan.
5. Gii thch c vai tr ca creatin phosphat v con ng o thi ca creatin v
creatinin.

1. I CNG

1.1. S tng qut (hnh 1)

Hnh 14.1. S chuyn ha protid

1.2. Nhu cu
Mi ngy c th cn 30 - 60 gam protein. Lng protid dng :
Tng hp protein cu trc v chc nng: collagen, myosin.
Tng hp protein c hot tnh sinh hc: enzym, mt s hormon.
Tng hp mt s cht c hot tnh sinh hc: histamin, serotonin
Cung cp 12% tng nng lng c th.
1.3. Ngun gc
- Acid amin ngoi sinh: do ng vt, thc vt v vi sinh vt nh to, men bia cung cp cho
c th qua qu trnh tiu ha v hp th protid.
- Acid amin ni sinh: tng hp c trong c th v do protein ca t bo b thy phn
thnh acid amin ta gi l cc acid amin ni sinh. Enzym thy phn c tn l catepsin (A, B,
C) c c tnh v c ch ging enzym peptidase. Cc catepsin ny nm trong lysosom, khi t
bo cht mng lysosom b ph v, chng c gii phng v tc dng ln protein t bo gy ra
hin tng t tiu.
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1.4. S thy phn protid ng tiu ha

Qu trnh ct t cc lin kt peptid nh cc enzym c tit ra t ng tiu ha (proteinase
- protease hay peptidase).

Hnh 14.2. S thy phn protein v peptid

Hai loi peptidase: endopeptidase v exopeptidase .

- Endopeptidase: pepsin, trypsin v chymotrypsin.
- Exopeptidase: c ba loi:
* Carboxypeptidase ca dch ty thy phn lin kt peptid cui chui polypeptide v pha
C tn, gii phng cc acid amin t do.
* Aminopeptidase ca dch rut, thy phn lin kt peptid tn cng c nhm amin u, gii
phng cc acid amin t do.
* Dipeptidase ca dch rut, thy phn cc dipeptid gii phng cc acid amin t do.
Mi loi peptidase c tc dng c hiu i vi v tr ca lin kt peptid v bn cht ca gc
R ca cc acid amin tham gia to thnh lin kt peptid ca chui protein. Lc u cc peptidase
trong dch v, dch ty v dch trng c tit ra di dng khng hot ng gi l proenzym
sau tr thnh hot ng trong ng tiu ha gip s thy phn protein (hnh 14.2).

Hnh 14.3. Pepsinogen v pepsin

Hnh 14.4. Enzym thy phn protein trong dch tiu ha

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1.3. S hp th cc acid amin
- Cc acid amin loi L c hp th theo c ch hp th ng vi s tham gia ca pyridoxal
phosphat.
- Cc acid amin loi D c hp th theo c ch khuch tn thng thng.
C hin tng cnh tranh hp thu khi n mt nhm nhiu acid amin.
Trong vi trng hp c bit, cc protein hay chui peptid ln c th c hp thu qua
mng rut m khng b thy phn, gy nn hin tng d ng hay mn cm i vi mt s thc
n nh tm, cua, c...
Mng rut tr c th hp thu c protein trng lng phn t tng i ln. Nh vy m
cc khng th ca m theo sa c hp thu gy tnh min dch cho tr.

2. THOI HA NIT CA CC ACID AMIN

* S tm tt qu trnh thoi ha chung ca acid amin (AA) (hay l cc ng chnh

trong sinh tng hp ur).

Hnh 14.5. Thoi ha chung ca acid amin

2.1. Phn ng chuyn amin: xc tc l aminotransferase (transaminase) c coenzym l

pyridoxal phosphat (vitamin B6)

Hnh 14.6. Phn ng chuyn amin

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Chng XIV: CHUYN HA LIPID

Transaminase c nhiu c, gan, tim, thn, rut cc m ng vt c hai transaminase

ph bin v hot ng mnh nht l glutamat oxaloacetat transaminase (GOT) v glutamat
pyruvat transaminase (GPT). Tt c cc acid amin u c th chuyn amin nhng vi mc
khc nhau, mnh nht l glutamat v aspartat. Sau n alanin, glycin, valintr vi acid
amin kh cho phn ng chuyn nh lysin, threonin, ornithin.

Hnh 14.7. Cc transaminase GPT v GOT

Hnh 14.8. Glutamat pyruvat transaminase (GPT) hay alanin amino transferase (ALT)

Hnh 14.9. Glutamat oxaloacetat transaminase (GOT) hay aspartat amino transferase (AST)

Khi tn thng gan, GOT v GPT tng cao trong huyt thanh, c bit GPT. Khi tn thng
tim, GOT v GPT tng cao trong huyt thanh, c bit GOT.
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2.2. Phn ng kh amin: l mt qu trnh quan trng trong nhm NH2 tch khi phn
t acid amin di dng NH3
- Kh amin oxy ha (hnh 10).

Hnh 14.10. Kh amin oxy ha

Gm hai giai on (hnh 11).

Hnh 14.11. Hai giai on ca s kh amin oxy ha

- Kh trc tip L - glutamat.

L - glutamat kh bi enzym L - glutamat dehydrogenase m coenzym l NAD+ c hot tnh
cao hot ng mnh nht pH = 7,3 thch hp vi pH ca c th nn kh nng kh amin trc
tip L - glutamat d dng nht.

Hnh 14.12. Kh amin oxy ha glutamat

- Kh gin tip (phn ng chuyn v kh amin).

Cc acid amin khc c enzym hot ng mi trng qu kim pH = 10 v coenzym FMN
(hay FAD) c hot tnh yu nn phi kh amin gin tip qua h thng chuyn amin.
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vn chuyn nhm

amin (hnh 14.13).

Hnh 14.13. Chuyn v kh amin

Trao i amin v kh amin oxy ha lun kt hp vi nhau, cc acid amin thng trao i
nhm amin vi - cetoglutarat to glutamat, sau glutamat dehydrogenase kh amin di
dng NH3.
- Phn ng amin ha.
L phn ng ngc li vi phn ng kh amin oxy ha, l s tng hp li acid amin t
acid - cetonic v NH3.
V d: Tng hp alanin t acid pyruvic v NH3 (hnh 14).

Hnh 14.14. S amin ha

2.3. S chuyn ch NH3

NH3 c to thnh cc m, ch yu do s kh amin oxy ha cc acid amin. N c
gn vo acid glutamic to thnh glutamin nh enzym glutamin synthethase (phn ng amid
ha).

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Hnh 14.15. S to thnh glutamin

Glutamin c vn chuyn theo s sau (hnh 14.16).

Hnh 14.16. Vn chuyn NH3

2.4. Chu trnh to thnh ur (chu trnh Ornithin ca Krebs v Henseleit)

Ur c to thnh gan qua nm giai on sau:

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Hnh 14.17. Chu trnh ur (giai on 1, 2 xy ra trong ty th,

giai on 3, 4, 5 xy ra bo dch ca t bo gan)

- Giai on 1: to thnh carbamyl phosphat do s gn NH3 t do vi CO2 nh s xc tc

ca carbamyl phosphat synthase trong khoang ty th (hnh 14.18).

Hnh 14.18. S to thnh carbamyl phosphat

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- Giai on 2: chuyn nhm carbamyl phosphat ti ornithin to thnh citrullin nh ornithin
carbamyl transferase ty th (hnh 14.19).

Hnh 14.19. S to thnh citrullin

- Giai on 3: to thnh arginosuccinat gan do s kt hp citrullin vi aspartat nh

arginosuccinat synthase vi s tham gia ca ATP (cung cp nng lng) v ion Mg++ (hot ha
enzym) xy ra bo dch (hnh 14.20).

Hnh 14.20. S to thnh arginosuccinat

- Giai on 4: phn ly arginosuccinat thnh arginin v fumarat nh arginosuccinat lyase

(arginosuccinase) xy ra ti dch bo ca gan (hnh 14.21).

Hnh 14.21. S to thnh arginin

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chu trnh Krebs, fumarat chuyn thnh L - malat ri thnh oxaloacetat v cht ny
bin thnh aspartat nh s amin ha hoc chuyn amin (hnh 22). Aspartat ng vai tr a
nhm NH3 th hai vo chu trnh ur.

Hnh 14.22. Fumarat bin thnh aspartat

- Giai on 5: to ur do arginase xc tc s thy phn arginin tch ur ra v ti to

ornithin (phn ng xy ra dch bo) (hnh 14.23).

Hnh 14.23.Thy phn arginin to ur

Phng trnh tng qut ca s tng hp ur l:

S tham gia ca ty th v bo dch vo chu trnh ur c th hin trong s sau (hnh
14.24).

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Hnh 14.24. Vai tr ca ty th v bo dch trong s to ur

Ri lon chuyn ha lin quan n cc phn ng ca chu trnh ur c th gp nh:

Tng amoniac mu kiu 1 (do thiu ht enzym carbamyl phosphat synthase).
Tng amoniac mu kiu 2 (do thiu ht enzym ornithin transcarbamylase = ornithin
carbamyl transferase).
Tng citrullin mu v nc tiu (do thiu ht enzym arginosuccinat synthase).
Tng arginosuccinat mu, dch no ty v nc tiu (do thiu ht enzym
arginosuccinase).
Tng arginin mu v dch no ty (t enzym arginase trong hng cu).

3. THOI HA NHM - COOH CA CC ACID AMIN (hnh 14.25)

Hnh 14.25. S kh carboxyl ca acid amin

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Hnh 14.26. Kh carboxyl ca histidin

Hnh 14.27. Kh carboxyl ca glutamat

Hnh 14.28. Kh carboxyl ca lysin

Cc amin v diamin c kh amin oxy ha nh monoamino oxydase hoc diamino

oxydase (hnh 14.30).

Hnh 14.29. Kh carboxyl ca tyrosin

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Hnh 14.30. Monoamino oxydase (MAO)

4. THOI HA SN CARBON (GC R) CA CC ACID AMIN

ng vt c xng sng c 20 h thng multienzym ca s thoi ha oxy ha qua chu
trnh Krebs i vi 20 acid amin (hnh 14.31).

Hnh 14.31. Thoi ha gc R ca acid amin

5. TNG HP ACID AMIN

ng vt c xng sng khng c kh nng tng hp 20 acid amin, chng ch c th tng

hp c mt s acid amin t cc sn phm chuyn ha ca glucid v lipid, l acid amin
khng cn thit.
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Cc acid amin c th khng tng hp c, phi ly t thc n (vi sinh vt v thc vt) a
vo c th, c gi l acid amin cn thit .
Bng 14.1. AA khng cn thit v AA cn thit

Acid amin cn thit

Arginin
Histidin
Isoleucin
Leucin
Lysin
Methionin
Phenylalanin
Threonin
Tryptophan
Valin

AA khng cn thit
Glycin
Alanin
Cystein
Acid glutamic
Glutamin
Acid aspartic
Asparagin
Tyrosin
Prolin
Serin

Gn y qua th nghim ca ROSE th arginin v histidin tr thnh hai acid amin bn cn

thit v ngi n l acid amin khng cn thit, chut v ng vt khc n l acid amin cn
thit.
C th ngi ch c th tng hp c 10 acid amin khng cn thit t acid amin khc
hoc t nhng sn phm thoi ha ca glucid, lipid.
- Glutamat c tng hp nh phn ng amin ha.

Hay phn ng thy phn glutamin di tc dng ca enzym glutaminase.

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- Glutamin c tng hp t glutamat + NH3 xc tc bi glutamin synthetase.

- Alanin v aspartat c tng hp t pyruvat v oxaloacetat nh phn ng chuyn amin t

glutamat.

- Asparagin c tng hp t aspartat v NH3.

- Serin c tng hp t 3 - phosphoglycerat.

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- Glycin c th c tng hp t nhiu ng khc nhau (nh t cholin, serin).

- Prolin c tng hp t glutamat.

- Cystein c th c tng hp t methionin v serin.

- Tyrosin c tng hp t phenylalanin nh phn ng hydroxyl ha.

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6. CHUYN HA CHUYN BIT CA MT S ACID AMIN

6.1. Acid amin to ng v acid amin to ceton (sinh ng hay sinh cht bo)

Hnh 14.32. Acid amin to ng

- Acid amin to ng: l cc acid amin c th chuyn ha thnh pyruvat,

- cetoglutarat succinyl - CoA, fumarat, oxaloacetat nh alanin, arginin, asparagin, aspartat,
cystein, glycin, glutamin, glutamat, methionin, histidin, prolin, threonin, valin.
- Acid amin to ceton: ch c leucin l acid amin n thun cho ceton ngha l bin thnh
aceto acetat.
- Acid amin to ng v to ceton trong qu trnh chuyn ha chng c th bin thnh
ng v thnh th ceton.
V d: isoleucin, lysin, phenylalanin, tyrosin, tryptophan (bng 14.2)
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Bng 14.2. Cc loi L - - AA to ng v to ceton

6.2. Glycin v serin

Glycin v serin l hai acid amin khng cn thit v l nhng acid amin sinh ng.
gan, serin bin i thnh glycin.

Hnh 14.33. Serin bin thnh glycin

Trong c th, glycin b kh amin oxy ha bi enzym glycin dehydrase cho acid glyoxylic
v NH3. Sau acid glyoxylic b oxy ha cho acid oxalic v kh CO2 thnh acid formic
(hnh 14.34).

Hnh 14.34. Glycin bin thnh acid formic

Trong chuyn ha, glycin tham gia cc qu trnh sinh tng hp lin quan n acid mt
(acid glycocholic), creatinin, hem, base purin.
6.3. Hydroxyprolin
Hydroxyprolin ca collagen c to thnh t gc prolin trong phn t collagen nh
phn ng hydroxyl ha (hnh 14.35).
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Hnh 14.35. Hydroxyl ha prolin

6.4. Histidin
S phn gii histidin bng s kh amin ha di tc dng ca histidase (hnh 14.36).

Hnh 14.36. Chuyn ha histidin

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6.5. Tryptophan
S phn gii tryptophan bt u bng s t vng indol bng cch oxy ha xy ra di tc
dng tryptophan oxygenase (hnh 14.37).

Hnh 14.37. Chuyn ha tryptophan

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Khi c th thiu acid nicotinic (tc thiu vitamin PP) s b bnh Pellagre da s sn si. Do
hm lng tryptophan trong thc n rt cn.
6.6. Phenylalanin v tyrosin
* S chuyn ha chnh ca phenylalanin v tyrosin (hnh 14.38).

Hnh 12.38. Chuyn ha phenylalanin v tyrosin

* Hydroxyl ha phenylalanin v tyrosin (hnh 14.39).

Hnh 14.39. Chuyn ha phenylalanin

* To thnh melanin (hnh 14.40).

Melanin l mt sc t mu m s phn phi v nng nh hng n mu da, thiu
sc t ny th b bnh bch tng (Albinism), ngi bnh c mu da trng. Melanin l mt cht
kh phc tp do s polyme ha cht 5-6 dihydroxyindol.

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Hnh 14.40. S to thnh melanin

* Tyrosin bin thnh acetoacetat v fumarat (hnh 14.41).

Hnh 14.41. Thoi ha tyrosin

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Trn ng chuyn ha trn nu thiu enzym homogentisat oxygenase th homogentisat
(hay Alcapton) li v bi tit ra nc tiu gy bnh Alcatonuria (Alccapton niu).
* To hormon gip trng (hnh 42).
T tyrosin to thnh cc hp cht co iod ca tuyn gip.

Hnh 14.42. Cu to ha hc ca hormon gip trng

T1, T2 l tin cht to T3, T4.

T3, T4 c hot tnh hormon gip trng.
MIT v DIT to T3 hot ng mnh nhng t: 3 - 5%.
DIT v DIT to T4 hot ng yu nhng nhiu: 35 - 40%.
S chuyn ha ca hormon gip trng (hnh 43).

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Hnh 14.43. S to thnh T3, T4

Nu con ng chuyn ha trn b ri lon, hormon gip trng b thiu dn n xut

hin bnh n n (tr con b chm pht trin v th lc v thn kinh).
* To thnh catecholamin (hnh 44).
Hormon ty thng thn gm adrenalin (epinephrin) v nor - adrenalin (nor epinephrin).

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Hnh 14.44. Chuyn ha catecholamin

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6.7. To thnh creatin

Qua 2 bc (hnh 45).

Hnh 14.45. S to thnh creatin

Creatin c chuyn qua mu ch yu ti c v tc dng vi ATP to nn creatin phosphat (mt dng d tr nng lng) (hnh 46).

Hnh 14.46. S to thnh creatin - phosphat

Khi tha ATP th nng lng c chuyn sang tch tr creatin - phosphat v khi cn (v
d: khi co c) th lin kt phosphat giu nng lng creatin - phosphat c chuyn sang ADP
to thnh ATP.
Creatinin l sn phm kh nc ca creatin trong c v c chuyn qua mu ti thn thi
ra nc tiu.

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6.8 To thnh glutathion

Qua hai bc (hnh 47).
Glutathion l mt tripeptid c tng hp gan.

Hnh 14.47. S to thnh glutathion

Glutathion tham gia qu trnh oxy ha sinh hc.

6.9. To thnh taurin: gan

Hnh 14.48. S to thnh taurin

gan cs kt hp taurin vi acid mt to nn cc dng mui mt cha taurin (v d:

taurocholat).

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CU HI T LNG GI
1. Enzym no sau y thy phn lin kt peptid u tin ca chui polypeptid:
A. Trypsin
B. Aminopeptidase
C. Chymotrypsinogen
D. Procarboxypeptidase
2. Sn phm chuyn ha acid amin o thi ra trong nc tiu ca ng vt di dng:
A. Acid uric
B. Ammoniac
C. Ur
D. Creatinin
3. Trong qu trnh to melanin t tyrosin cn c enzym no tham gia:
A. Tyrosin hydroxylase
B. Dopa hydroxylase
C. Diamin oxidase
D. Peroxidase
4. Cc cht sau y l sn phm chuyn ha ca histidin, NGOI TR:
A. Urecanat
B. Leucin
C. N - formimino glutamat
D. L - glutamat
5. Qu trnh bin i alanin thnh ng c gi l:
A. ng phn
B. Tn to glucose
C. Kh CO2 oxy ha
D. Kh amin oxy ha
6. Acid amin no sau y khng tham gia chu trnh ur:
A. Arginin
B. Citrullin
C. Histidin
D. Ornithin

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7. Trong chu trnh ur, giai on no cn c s tham gia ca ATP:
A. Giai on 1 v 2
B. Giai on 1 v 3
C. Giai on 1 v 4
D. Giai on 3 v 4
8. Tp hp no c mt trong chu trnh ur:
A. Carbamyl , ornithin, arginin, acetyl CoA
B. Carbamyl , ornithin, citrullin, L - malat
C. Ornithin, citrullin, arginosuccinat, arginin
D. Carbamyl , asparagin, ur
9. Acid amin khng cn thit c c th:
1. T tng hp m khng cn cung cp theo ng thc n
2. Tng hp theo con ng ring ca tng acid amin
3. Tng hp nh cc enzym trong phc hp multienzym
4. Tng hp c t cc - ceto acid tng ng
Chn tp hp ng:
A.1, 3, 4
B. 1, 2, 3
C. 1, 2, 4
D. Tt c cc cu trn u ng
10. Phn ng to - cetoglutarat t L - glutamat c gi l:
A. Kh amin
B. Chuyn amin
C. Kh CO2 oxy ha
D. Kh amin oxy ha
11. Protein no sau y khng b enzym thy phn trong ng tiu ha:
A. Myosin
B. Globulin
C. Keratin
D. Serotonin
12. Enterokinase hot ha enzym no sau y:
A. Trypsin
B. Trypsinogen
C. Elastase
D. Pepsin
13. Transaminase trong phn ng chuyn amin thng c nhiu :
A. Cc m ng vt
B. Trong th ty
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Chng XIV: CHUYN HA LIPID

C. Bo dch
D. Nhn t bo
14. Trong giai on tiu ha protein:
A. Cc enzym thy phn tc dng c tnh c hiu i vi v tr ca cc lin kt peptid
B. C s tham gia ca phn t H2O trong phn ng ct cc lin kt peptid
C. Chu tc dng ca cc enzym: pepsin, trypsin, chymotrypsin, carboxypeptidase,
aminopeptidase
D. Tt c cc cu trn u ng
15. Qu trnh kh amin oxy ha ca acid amin xy ra qua 2 bc, mt cht trung gian
c to ra l:
A. - cetoacid
B. Ur
C. - imin acid
D. Acid uric
16. Phn ng kh CO2 ca acid amin no sau y to ra 1 cht lm dn mch:
A. L - acid glutamic
B. L - arginin
C. L - acid aspartic
D. L -histidin
17. Phn ln ammoniac c phng thch ra thn l t acid amin no sau y:
A. Leucin
B. Glutamin
C. Glycin
D. Alanin
18. Acid amin cn thit:
A. C th khng th tng hp y m phi c cung cp bi thc n
B. Tng hp thn nhng khng c to ra cc m khc
C. Khng c bi tit v qu quan trng i vi c th
D. c bin dng bi men D- acid amin oxidase
19. Trong no c 1 enzym bin i acid glutamic thanh acid - aminobutyric v phn
ng ny i hi s hin din ca cht no sau y:
A. ATP
B. Serin
C. Carbon dioxide
D. Pyridoxal phosphat
20. Trong cc ng vt thi ra ur, nhm carbamyl c chuyn qua ornithin to ra:
A. Pyrimidin
B. Ur
C. Citrullin
D. Arginin
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21. Trn ng bin dng ca L - tyrosin, mt hormon c to thnh c tc dng lm:
A. H huyt p
B. m hi
C. Tng huyt p
D. Albinism
22. Nhm methyl gn vo cystein to thnh:
A. Methionin
B. Cystin
C. Ethanolamin
D. Acid cysteic
23. Thyroxin (T4) dn xut t:
A. Tyramin
B. Taurin
C. Tryptamin
D. Tyrosin
24. Albinism l bnh l bm sinh do thiu enzym:
A. Homogentisicase
B. Phenylalanyl hydroxylase
C. Tyrosinase
D. Tyrosin decarboxlase
25. Alkaptonuria l bnh l bm sinh do thiu enzym:
A. Tyrosinase
B. Phenylalanin hydroxylase
C. Homogentisic acid oxidase
D. DOPA decarboxylase
26. Phenylketonuria l bnh l bm sinh do thiu enzym :
A. Kh amin ca phenylalanin
B. Hydroxy ha phenylalanin
C. Kh carboxyl ca phenylalanin
D. Bin i tyrosin thnh DOPA

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CU HI NGHIN CU BI HC
1. Gan l c quan duy nht tng hp albumin v ur?
Khi t bo gan suy th albumin v ur mu u gim?
Trong 2 xt nghim trn th xt nghim no hay dng nh gi chc nng tng hp ca
t bo gan?
2. Trong cc enzym tiu ha protid, enzym no hot ng mi trng acid? Enzym no
thy phn lin kt gia cc acid amin kim? acid amin c nhn thm?
3. K tn 2 enzym chuyn nhm amin hot ng mnh nht trong c th? Hai enzym ny
thng dng chn on bnh g trong lm sng? Trong bnh l no hai enzym ny
thay i nh th no?
4. Nu ngun gc ca amoniac mu? Trong mu phn ln amoniac gn kt vi cht g lu
thng trong tun hon?
K tn sn phm o thi ca amoniac gan v thn?
5. Nu tn cc c quan tham gia sinh tng hp creatin trong c th? Creatin c nhiu u
v di dng gn kt g?

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