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1

METABOLISMUL AMINOACIZILOR
INDIVIDUALI
Glicocol
Serin
Aminoacizi cu sulf: cistein, cistin, metionin
Aminoacizi aromatici: fenilalanin, tirozin
2
METABOLISMUL GLICOCOLULUI
H
2
C COOH
NH
2
Aminoacid NEESENIAL
Aminoacid GLUCOFORMATOR
Structura:

METABOLISMUL GLICOCOLULUI
I. BIOSINTEZ
1) Din SERIN

COOH
NH
2
OH NH
2
COOH
(-CH
2
-OH)
H
2
C H
2
C
CH
Serin
Glicocol
PLP
H
2
O
FH
4
N
5
- N
10
- CH
2
- FH
4
serin-hidroximetil
transferaza
+
2) Din TREONIN

OH
H
2
N
COOH
O
CH
3
-COOH
COOH
NH
2
CH
3
HC
CH
L-Treonin
CH
3
CH
Acetaldehid
H
2
C
Glicocol
treonin
aldolaza
(clivarea
moleculei)
3
Serina i treonina sunt singurii aminoacizi care formeaz glicocol
3) Din SARCOZIN

COOH
NH
COOH
NH
2
H
2
C
CH
3
Sarcozin
(N-metil glicocol)
CH
3
H
2
C
Glicocol
II. TRANSFORMRILE GLICOCOLULUI

1)Precursor n biosinteza SERINEI
2)Precursor n biosinteza ETANOLAMINEI (prin intermediul serinei)
3)Precursor n biosinteza COLINEI
4)Precursor n biosinteza SARCOZINEI (metilare cu SAM, n muchi)
5)Precursor n biosinteza CREATINEI (alturi de ARGININ i
METIONIN)

4
Sarcozina compus care
se gsete n muchi, i
este utilizat n industrie la
sinteza surfactanilor
biodegradabili i a pastelor
de dini
HOOC NH
O
CH
SH
NH
O
NH
2
COOH
CH
2
C
CH
2
C
CH
2
CH
2
HC
6 ) Rol n biosinteza GLUTATIONULUI: tripeptid
Glicocol Cistein Acid glutamic

7) n biosinteza nucleotidelor PURINICE:

N
H
N
N
N
1
2
3
4
5
6
7
8
Molecula de glicocol este folosit pentru a forma poziiile 4, 5, 7
din scheletul purinic
5
Biosinteza se face cu consum de ATP
8 ) n biosinteza nucleului PIROLIC
Glicocolul SINE QUA NON

N
Hemoglobina
Clorofila
Citocromii
9) n funcia de detoxifiere a ficatului
Conjugarea glicocolului cu acid benzoic

COOH
NH
2
COOH
O
NH CH
2
-COOH
H
2
C
Glicocol
+
C
Acid hipuric
H
2
O
Determinarea cantitativ, n urin, de acid hipuric este utilizat ca test
pentru explorarea funciei hepatice - marker de toxicitate).

10) n formarea ACIZILOR BILIARI CONJUGAI
GLICOCOL + Acid colic Acid glicocolic

6
11) Dezaminarea oxidativ catabolism propriuzis

COOH
NH
2
COOH
O
HCOOH
COOH
COOH
H
2
C
Glicocol
glicinoxidaz
FMN
HC
Acid glioxilic
sau
7
Hiperoxalurie primar
METABOLISMUL SERINEI
Structura:

COOH
NH
2
OH
HC H
2
C
- aminoacid neesential glucoformator
- acid - amino - - hidroxi propionic
- hidroxi aminoacid
Biosinteza serinei
Din glicocol

Din acid 3-fosfogliceric,
(intermediar n glicoliz)
Biosinteza din glicocol (INTERCONVERSIA):
COOH
NH
2
COOH
NH
2
OH
H
2
C
Glicocol
+ N
5
- N
10
- CH
2
- FH
4
+ PLP + H
2
O
(-CH
2
OH)
HC H
2
C
Serin
serin-hidroximetiltransferaza
8
Silk protein
Biosinteza
din acid 3-fosfogliceric:
COOH
HO
O PO
3
H
2
COOH
OH
O PO
3
H
2
COOH
O
O PO
3
H
2
COOH
O
OH
COOH
OH
H
2
N
COOH
H
2
N
O PO
3
H
2
COOH
OH
H
2
N
CH
CH
2
Acid L - 3- fosfogliceric
HC C
CH
2
CH
2
Acid 2-fosfogliceric Acid 2-ceto 3-fosfogliceric
izomerizare
NAD
+
NADH + H
+
P
i
C
CH
2
Acid -cetogliceric
Alanin
Acid piruvic
L - Serin
Fosfo L - serin
CH
CH
2
NAD
+
NADH + H
+
CH
CH
2
L - Glutamat
Acid -cetogutaric
P
i
+ H
2
O
L - Serin
CH
CH
2
transaminare
transaminare
oxidare
defosforilare
oxidare +
defosforilare
9
TRANSFORMRILE SERINEI

1. Biosinteza COLINEI Lecitine

COOH
OH
H
2
N
OH
NH
2
OH
NH
CH
3
OH
N
CH
3
CH
3
OH
N
CH
3
CH
3
CH
3
CH
CH
2
L - Serin
PLP
CO
2
H
2
C
CH
2
Etanolamin
+ "CH
3
"
SAM
FH
4
H
2
C
H
CH
2
N - metil
etanolamin
+ "CH
3
"
H
2
C
H
CH
2
N - dimetil
etanolamin
+ "CH
3
"
H
2
C
H
CH
2
N - trimetil
etanolamin
(COLIN)
+
10
2. Biosinteza SARCOZINEI i a GLICOCOLULUI Ciclul serinei
OH
N
CH
3
CH
3
CH
3
O
N
CH
3
CH
3
CH
3
N
CH
3
CH
3
CH
3
N
CH
3
CH
3
NH
CH
3
NH
2
H
2
C
CH
2
COLIN
+
oxidare
H
2
C
HC
+
Betainaldehid
oxidare
H
2
C
COOH
+
Betain
demetilare
CH
3
H
2
C
H
COOH
N - dimetil
glicocol
demetilare
CH
3
H
2
C
COOH
N - metil
glicocol
(SARCOZIN)
demetilare
CH
3
H
2
C
COOH
Glicocol
(-CH
2
OH)
FH
4
, PLP
SERIN
3. Biosinteza ACIDULUI PIRUVIC Glicoliz
COOH
OH
H
2
N
COOH
O CH
H
CH
2
L - Serin
PLP
serin-deshidrataza
C
CH
3
Acid piruvic
H
2
O + NH
3
Metabolism
GLUCIDIC
(aminoacid
glucoformator)
11
12
+ Colinesteraza
+ Ciclooxigenaza

13
antibiotic + antituberculos
14
AMINOACIZI CU SULF

COOH
NH
2
SH
COOH
NH
2
S S CH
NH
2
HOOC H
2
N
COOH
S
(CH
2
)
2
HC H
2
C
HC H
2
C CH
2
Cistein
Cistin
L- Metionin
CH
3
aminoacid semiesential
aminoacid esential
CH
15
I. Biosinteza CISTEINEI
din ali aminoacizi

H
2
N
COOH
S
(CH
2
)
2
N
N
N
N
NH
2
S
H
2
N
COOH
(CH
2
)
2
ATP
O
OH OH
"CH
3
"
H
2
N
COOH
(CH
2
)
2
SH
CH
CH
3
+
H
2
C
CH
3
CH
+
P
i
+ PP
i
Sulf-adenozil
metionin (SAM)
Sulf-adenozin
homocistein
L- Metionin
Adenozin
CH
L- Homocistein
S-adenozil
metionin
sintetaza
16
H
2
N
COOH
(CH
2
)
2
SH
HO
H
2
N
COOH
CH
2
H
2
N
COOH
CH
2
S
NH
2
COOH
CH
2
H
2
N
COOH
CH
2
SH
H
2
N
COOH
CH
2
OH CH
2
CH
L - Homocistein
+
CH
L - Serin
H
2
O
CH
Cistationin
HC
cistationinaza
CH
L - Cistein
+ H
2
O
CH
L - Homoserin
cistationin
sintetaza
PLP
PLP
Aminoacid semiesenial
reacie de
condensare
Marker de disfuncie
endotelial
17
II. Transformrile CISTEINEI

1) Dezaminare desulfurant

COOH
CH
2
SH
O
COOH
CH
3
O H
2
N
COOH
CH
2
SH
NH
3
PLP
cistein
desulfhidraza
+ H
2
O
C
Acid -mercapto
piruvic
H
2
S
C
Acid piruvic
Metabolism
GLUCIDIC
(aminoacid glucoformator)
CH
L - Cistein
DEGRADRILE:
18
2) Decarboxilare

NH
2
CH
2
SH
CH
2
H
2
N
COOH
CH
2
SH
CO
2
PLP
Cisteamin
ACP
HSCoA
CH
L - Cistein
3) Oxidare blnd: 2 CISTEIN CISTIN

NH
2
COOH
NH
2
COOH
CH CH
2
CH CH
2
S
S
NH
2
COOH
H
CH
2
S H C
Acyl Carrier Protein
-2H
+2H
-n celulele tinere: -SH
-n celulele senescente: -S-S-
19
H
2
N
COOH
CH
2
SO
2
H
H
2
N
COOH
CH
2
SO
3
H
NH
2
CH
2
SO
3
H
CH
2
H
2
N
COOH
CH
2
SH
Acid L-cistein
sulfinic
oxidare
CH
oxidare
CH
Acid L-cisteic
CO
2
Taurina
+ Colil~SCoA
ACID TAUROCOLIC
CH
L - Cistein
4) Oxidare intens: formarea TAURINEI

+ acid GLICOCOLIC
Acizi biliari conjugai
Taurina - Larg rspndit n esuturi, se gsete n
bil, esenial pentru funcia CV, nervoas, se
gsete n fructele de mare
(ac. 2-amino-
etansulfonic)
20
5) Cisteina GLUTATION
6) CISTEINA i CISTINA Rol plastic stabilizarea moleculei
proteice
Structura a numeroase polipeptide i proteine (insulin,
scleroproteine, fibrinogen)

7) CISTEINA Structura a numeroase enzime -
centrul catalitic

8) n reacii de DETOXIFIERE:
Sub form de N-acetilcistein n procesul de detoxifiere al
derivailor aromatici halogenai (genereaz acizi mercapturici)

9) n reacii de TRANSFER al gruprilor SULFAT ( surs de SO
4
2-

pentru sulfotransferaze)

21
n concluzie, deficienele n metabolismul metioninei pot
avea consecine mult mai severe n sinteza proteic dect
oricare alt aminoacid

prin forma sa activ SAM
22
METABOLISMUL AMINOACIZILOR AROMATICI
CH
2
CH N H
2
COOH
CH
2
CH N H
2
COOH
OH
C
H
2
C
H
NH
2
COOH
O H
O H
C
H
2
C
H
2
NH
2
O H
C
H
C
H
2
NH
2
O H
OH
C
H
2
O
COOH
O H
CH
2
O
COOH
OH
OH
CH
2
COOH
OH
OH
CH
2
CH
2
NH
2
CH
2
O
COOH
Fenilalanina
Fenilalanin
hidroxilaza
Tirozina
Biosinteza hormonilor tiroidieni
Tirozin
hidroxilaza
Dioxifenil
alanina (DOPA)
Hormoni medulosuprarenali
(adrenalina, noradrenalina)
Melanine
(hormoni responsabili de pigmentarea pielii)
CO
2
Tiramina
(efect de cretere a presiunii arteriale)
Octopamina
Hidroxilare
la catena
lateral
Acid ceto glutaric
Acid glutamic
Transaminare
Acid p-hidroxifenil piruvic
2-hidroxilaza
Acid 2,4 dihidroxifenil piruvic
Decarboxilare
Acid homogentizic
Homogentizat
oxidaze Acid fumaric
+
Acid acetic
Decarboxilare
Fenil etil
amina
Transaminare
Acid fenilpiruvic
Acid fenil acetic
Acid fenil lactic
Toxici ai celulei nervoase
1
a
b
c
d
2
23
C H
2
CH
NH
2
COOH
FENILALANINA = aminoacid esenial
sau
CH
2
CH N H
2
COOH
L- Fenilalanina
24
1. Hidroxilarea fenilalaninei => TIROZIN
CH
2
CH N H
2
COOH
CH
2
CH N H
2
COOH
OH
L- Fenilalanin
L-Tirozin
fenilalanin 4-hidroxilaza
acid ascorbic
+ O
2
citP450
NADPH
2
NADP
+
H
2
O
a). Biosinteza hormonilor tiroidieni

Tirozin
Tironin Tiroxin (T
3
)
(aminoacid)
(intemediar)
(hormon)
25
CH
2
CH N H
2
COOH
OH
C
H
2
CH
NH
2
OH
OH
COOH
L-Tirozin
3,4-Dihidroxifenilalanin
(DOPA)
tirozin-hidroxilaza
1
2
3
4
5
6
b.) Hidroxilarea tirozinei
Hormoni
medulosuprarenali:
adrenalina
noradrenalina
Melanine = Hormonii
responsabili de
pigmentarea pielii i a
prului, n melanocite
Dopamin
26
c.) Decarboxilarea tirozinei
CO
2
CH
2
CH N H
2
COOH
OH
C
H
2
CH
OH
NH
2
OH
C H CH
OH
NH
2
PLP
L-Tirozin
Tiramina
tirozin-decarboxilaza
hidroxilare la catena
laterala
Octopamina
(neuromediator)
Descoperit la caracati
(1948)
La lcuste- rol n contracia
muscular
amin biologic activ
(hormon tisular)
27
d.) Transaminarea tirozinei = cale de eliminare
COOH
CO
2
OH
C
H
2
OH
COOH
OH
CH
2
OH
Acid 2,4-dihidroxifenil
piruvic
C O
PLP
decarboxilare
Acid
homogentisic
Acid fumaric
+
Acid acetil
acetic
Homogentizat
oxidaz
CH
2
CH N H
2
COOH
OH
CH
2
OH
COOH
CH
2
OH
OH
COOH
L-Tirozin
+ Acid -cetoglutaric
Acid glutamic
PLP
Acid
p-hidroxifenil
piruvic
C O
Acid
p-hidroxifenil
lactic
C H
2-hidroxilaza
Ciclu Krebs
28
CH
2
CH N H
2
COOH
CH
2
CH
2
NH
2
CO
2
CH
2
COOH
NH
3
L- Fenilalanin
Feniletilamin
C O
Acid
fenilpiruvic
Acid fenil acetic
Acid fenil lactic
2. TRANSFORMAREA
FENILALANINEI N
METABOLII TOXICI PENTRU
CELULA NERVOAS
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