curs biofarmacie

8/13/2019 Curs biofarmacie

1/256

LUCIANA DOBJANSCHI

BIOCHIMIE FARMACEUTIC

EDITURA UNIVERSITII DIN ORADEA


2/256

2007


3/256

Biochimie farmaceutic

Refereni tiinifici:

Conf.dr. !re"n "ri"n"Conf.dr. ic#e Oti#i"

EDITURA UNIVERSITII DIN ORADEA ESTE ACREDITAT DECNCSIS

CONFORM POIIEI !"#$

Ti%ar&METROPO'IS SR'

Ora(ea) *tr$ Nico+ae ,i-a .r$/!Te+$& 012# 3"4 !50) 0133 3"4 !50

Te+$6 Fa7& 024# "12 5"0e8mai+& metro%o+i*9r(*+i.:$ro

$

Descrierea CIP a Bibliotecii Naioale a Ro!"ieiDOBJANSCHI# LUCIANA Bioc$i!ie %ar!ace&tic'% Do&'"n(c)i *!ci"n". +Or"de" : Edit!r" Uni,er(it-ii din Or"de" 2007

/iior. IS/N 17+17$+731+422+

377.5:653


4/256

'ucia.a Do;


5/256


4II(7( Utili+area sc$elet&l&i )e ato!i )e carbo ai a!ioaci+ilor4II(8( Biosite+a a!ioaci+ilor4III( Metabolis!&l &cleoti)elor

4I/( Metabolis!&l $e!o,lobiei4/( Biblio,ra%ie

3


6/256

'ucia.a Do; cercet-ri ce "! de8on(tr"t c- "ce(te roe8e f!nd"8ent"#eot fi eficient "&ord"te rin 8etode &ioc)i8ice.

?ntr+"de,-r (!cce(!# &ioc)i8iei


7/256


"nor"nic-. D"c- &io#oi" e(te c)i8ie e" e(te !n fe# de s&*rac$i!iec"re inc#!de d"r


8/256

'ucia.a Do;


9/256


T"&e#!# 2. ;rinci"#e#e &io8o#ec!#e din or"ni(8!# !8"n

/io8o#ec!#" Unit-i (tr!c!r"#e !nciiADN de9o=iri&on!c#eotide "teri"# eneticARN ri&on!c#eotide ode# entr!

(inte9" roteine#orroteine "8ino"ci9i !ncii 8!#ti#e#!cide 8ono9")"ride Re9er,- de enerie#iide Aci9i r"i i "#i

co8oneni!ncii 8!#ti#e

Co!*oeii !ieralire9eni


10/256

'ucia.a Do;


11/256


T"&e#!# $. Di(tri&!i" c"tioni#or intr"+ i e=tr"ce#!#"r


12/256

'ucia.a Do;


13/256


/"9e#e iri8idinice re9int- !n n!c#e! de &"9- n!8it iri8idin" de#" c"re deri,- $ &"9e iri8idinice c"re int-


14/256

'ucia.a Do; ri&o9" 2 > de9o=i > D > ri&o9"

54


15/256


;rin "t""re" !nei ento9e #" &"9" "9ot"t- re9!#t- on!c#eo9id- c"re de9o=i"deno9in" "deno9in"

;rin "t""re" !nei 8o#ec!#e de "cid fo(foric #" (tr!ct!r" !nein!c#eo9ide (e for8e"9- !n n!c#eotid.

N

N

N

N

N=2

2=

O==

O=

C

=

=

=

OP

O=

O

O

PO

O

O=

P

O=

O

=O

AMP

ADP

ATP

53


16/256

'ucia.a Do; ri&o9".

2= O==

O=

C

=

=

=

OP

O=

O

=O;a>a a>otat?

de9o=iri&on!c#eotid

2=O

==

O=

C

=

O=

=

OP

O=

O

=O;a>a a>otat?

ri&on!c#eotid

56


17/256


N!c#eo9idtrifo(f"ii re9int- o (erie de f!ncii i8ort"nte.AT; e(te !n tr"n(ortor de r!-ri fo(f"t i irofo(f"t !#!i e(te refo(fori#"t #"AT;


18/256

'ucia.a Do; !# e(te co8!( din #"n!ri deri&on!c#eotide. Aci9ii de9o=iri&on!c#eici i "ci9ii ri&on!c#eicire9int- o (erie de rorit-i fi9ice i c)i8ice co88!ne )idro=i# " !r8-to"rei n!c#eotide.

5


19/256


O

C

=

= =

O

=

=

=2

;a>? a>otat?

4@

/@

P O=O

O

C=2O

=

==

=

;a>? a>otat?

O

P O=O

O

4@

/@

ADN

51


20/256

'ucia.a Do;? a>otat?

4@

/@

P O=O

O

C=2O

=

==

O=

;a>? a>otat?

O

P O=O

O

4@

/@

ARN

A(tfe# c"ten" rinci"#- " ADN i ARN con(t-


21/256


o#ec!#e#e de ADN din diferite ce#!#e i ,ir!(!ri (edeo(e&e(c rin r"ort!# dintre ce#e 4 ti!ri de 8ono8eri n!c#eotidicirin (ec,en" n!c#eotide#or i rin 8"(" 8o#ec!#"r-. ;e #n- ce#e 4

ti!ri de &"9e 8"'ore


22/256

'ucia.a Do;10 de n!c#eotide. iec"re dintre cei 20 de "8ino"ci9i din roteine "re

ce# !in !n ARNt coer(!n9-tor i"r !nii "! c)i"r 8"i 8!#i ARNt.De e=e8#!: citidi# >"deni# CCAB. r!"re" 3H>)idro=i# " "cid!#!i "deni#ic ter8in"# e(te

#e"t- de r!"re" $

H

>)idro=i# " "cid!#!i citidi#ic recedent rint+o!nte fo(fodie(teric-. r!"re" )idro=i# #i&erdin "cid!# "deni#icter8in"# e(te "ci#"t- en9i8"tic c! M+"8ino"cid!# (ecific for8nd"8ino"ci# >ARNt. Ace(t "8ino"cid e(te tr"n(fer"t en9i8"tic #"c"-t!# #"n!#!i o#ietidic


23/256


dect ce#!#e#e roc"riote e=i(t- 4 ti!ri de ARNr: 3S 7S 5S i 2S.Dei ARNr rere9int- o 8"re "rte din ARN ce#!#"r tot"# f!nci" #!i roteine c! (tr!ct!ri i f!ncii &io#oice fo"rteco8#e=eL ri&o9o8ii i ,ir!(!ri#e (!nt ce#e 8"i c!no(c!te. Ce#e 8"ico8#e=e (i(te8e "cid n!c#eic > rotein- (!nt ro&"&i# cro8o9o8iidin ce#!#e#e e!c"riote.

Ri&o9o8ii (!nt "rtic!#e ri&on!c#eoroteice c"re (e -(e(c


24/256

'ucia.a Do;tr"n(8i-tor.A8ino"ci9ii (!nt rec!r(orii !nei ,"rit-i de &io8o#ec!#e dee=e8#!: )itidin" entr! )i(t"8in-B. Unii "8in"ci9i (!nt8et"&o#i9"i i !ti#i9"i entr! rod!cere" de #!co9-#!coneoene9-B. Deo"rece n! e=i(t- re9er,e de "8ino"ci9i c!e=cei" ce#or i8#ic"i


25/256


R C= COO

N=/

8

?n toi M+ "8ino"ci9ii c! e=cei" #icinei c"r&on!# M e(te otic"cti,. I9o8erii o(i&i#i ""rin (eriei D ("! *. S+" con(t"t"t c- toii9o8erii e=i(teni


26/256

'ucia.a Do;


27/256


+ "8ino"ci9i c! do!- r!-ri &"9ice

=2N C=2 C=2 C=2 C=2 C= COO=

N=2

#i(in" #(B

=2N C N= C=2 C=2

N=

C=2 C= COO=

N=2"rinin" "rB

N N=

C=2 C= COO=

N=2

)i(tidin" )i(B

+ "8ino"ci9i "ro8"tici

C=2 C= COO=

N=2

feni#"#"nin" )eB

C=2 C= COO=

N=2

=O

tiro9in" trB

27


28/256

'ucia.a Do; c"re "!

r"dic"#!# o#"r d"r #i(it de ("rcin- e#ectric- #" 7.+ "8ino"ci9i )idrofi#i c! c"r"cter "cid: A( #! Tr > c"re (!nt

"8ino"ci9i "8ino"ci9i

c"re #" fi9io#oic (!nt


29/256


+ "cid!# K+ "8ino&!tiric A/AB e(te !n 8edi"tor c)i8ic ner,o(+ $4 > dio=ifeni#"#"nin" DO;AB e(te rec!r(or "# "dren"#inei+ "cid!# + "8ino&en9oic intr-


30/256

'ucia.a Do;


31/256


=OOC C=

N=2

C=2 C=2 CO N= C= CO

C=2

N= C=2 COO=

S=

#!t"tion!# (e


32/256

'ucia.a Do;ima (e co.Ger*i

A.-iote.*i.a II

octa%e%ti(

ami.o%e%ti(a>a

A.-iote.*i.a III

he%ta%eti(

%rotei.a %+a*matic?ca%to%ri+

Anioten(ine#e "cione"9- "(!r" ,"(e#or 8"i inten( e "rtere(ti8!#nd (ecrei" de "#do(teron i e#i&er"re" de ro(t"#"ndine.Anioten(in" II e(te (ocotit- ce# 8"i !ternic "ent re(or re(ent


33/256


Un "#t e=e8#! de o#ietide n"t!r"#e e(te rere9ent"t de,"(ore(in- i o=itocin- c"re (!nt etide cic#ice "#c-t!ite n!8"i din"8ino"ci9i. Ace(te do!- o#ietide "! o (tr!ct!r- fo"rte "roi"t-

fiind "#c-t!ite din 1 re(t!ri de "8ino"ci9i d"r re9int- f!nciifi9io#oice diferite. V"(ore(in" "re efect "ntidi!retic i"r o=itocin""cione"9- "(!r" 8!(c!#"t!rii netede " !ter!#!i rod!cndcontr"ci" !ter!#!i. O=itocin" (ti8!#e"9- i contr"ci" 8!(c!#"t!riidin '!r!# "#,eo#e#or 8"8"re deter8innd "(tfe# e#i8in"re" #"te#!idin c"n"#e#e #"ndei 8"8"re ",nd "ci!ne #"ct"o-.

$$


34/256

'ucia.a Do;


35/256


"(" 8o#ec!#"r- " roteine#or (e o"te deter8in" rin 8etodefi9ice i o"te fi c!rin(- rotein" din "#&!!# de o!+ c"9ein" > rotein" din #"te+ feritin" > rotein" de deo9it"re " e rotein" din r!

$. ;roteine de tr"n(ortE=e8#!:

+ )e8o#o&in" > rotein" ce tr"n(ort- o=ien!# rotein" ce tr"n(ort- "ci9ii r"i roteine#e din ci#i i f#"e#i.?n 8!c)i "ctin" i 8io9in" (!nt "e9"te


36/256

'ucia.a Do;


37/256


+ e#"(tin"+ 8!coroteine#e.

Aticor*ii -i r's*&s&l i!&( S*eci%icitatea )e s*ecie a*roteielor.

Anticorii ("! i8!no#o&!#ine#e "! o i8ort"n- fo"rte 8"re


38/256

'ucia.a Do;


39/256


i. 2. ;rinci"#e#e ti!ri de (tr!ct!ri "#e roteine#or

Str!ct!ri#e roteice (!nt (t"&i#i9"te de do!- ti!ri de #e-t!ri!ternice:legtura peptidic i puntea disulfidic rec!8 i de trei ti!ri de#e-t!ri (#"&e.*e-t!r" di(!#fidic- (e (t"&i#ete


40/256

'ucia.a Do; "8inic- " #i9inei.

Un ro# (e8nific"ti,


41/256


+ identific"re" "8ino"ci9i#or co8oneni rin difr"cie c! r"9e @ i(ectro(coie


42/256

'ucia.a Do;


43/256


Con(ecine#e for8-rii "ce(t!i (i(te8 !ternic con'!"t (!ntco#"n"rit"te" ce#or 4 "to8i cee" ce re(!!ne i8o(i&i#i"te" rotiriii e=i(tenei for8e#or ci( i tr"n(. S+" o&(er,"t c- i9o8er!# tr"n( e(te

f",ori9"t deo"rece e(te 8"i (-r"c


44/256

'ucia.a Do;


45/256


i!r" nr.3. Str!c!r" de fo"ie #i"t-.

A8ino"ci9i 8ici: # "#" ","nt"'e"9- fo"i" #i"t- i"r cei c!r"dic"# 8"re ("! c! ("rcin- (tn'ene(c "r"n'"8ent!#.

C" i 8ode#!# de M+)e#i= (tr!ct!r" P (e


46/256

'ucia.a Do;


47/256


3. Confor8"i" de "n("8! " #"n!#!i o#ietidic e(te ""rent"cee"i #" 8io#o&in" t!t!ror (ecii#or (ecii#or (t!di"te dei e#edifer- o"rec!8


48/256

'ucia.a Do;


49/256


?n 8o#ec!#" )e8o#o&inei r-8ne o c",it"te cent"#- 8-rinit- der!-ri c! r"dic"# R o#"re.

i!r" nr.. or8"re" )e8o#o&inei

Deat&rarea *roteielorDen"t!r"re" roteine#or rere9int- ert!r&"re" (tr!ct!rii #or

n"ti,e


50/256

'ucia.a Do;


51/256


/( /ITAMINE

Vit"8ine#e (!nt &io8o#ec!#e indi(en("&i#e ,ie!ito"re#or

entr! cretere"


52/256

'ucia.a Do;


53/256


/(0( /ita!iele li*osol&bile

Vit"8ine#e #io(o#!&i#e (!nt 8o#ec!#e "o#"re deri,"te din

i9oren. E#e (!nt re#"ti, (t"&i#e #" te8er"t!r" oer"ii#or c!#in"red"r !or in"cti,"te de r"di"ii#e UV i (!&(t"ne#e o=id"nte.Die(ti" i "&(or&i" #or dec!re


54/256

'ucia.a Do; c"roten

?n 8!co"(" inte(tin"#- P+ c"roten!# (!fer- o (cind"reo=id"ti,- " #e-t!rii d!e din o9ii" 53 roce( en98"ticB a

,it"8in" A5retin"#!#B

Tot


55/256


C=O

C=2O=

a+coo+

(ehi(ro-e.a>a

retino#

+ o=id"re" r!-rii "#de)idice #" "cid c"r&o=i#ic


56/256

'ucia.a Do;


57/256


Retial&lRetin"#!# "rtici- direct


58/256

'ucia.a Do;omeri>are

+umi.a

!!8ci* reti.a+

C= N=

o%*i.a

a++ tra.* reti.a+

;rin i9o8eri9"re (e (c)i8&- eo8etri" (tr!ct!rii o#ienice "rodo(inei "re #oc o de#"("re " #e-t!rii "#di8inice c! 3A0


59/256


;roce(!# de e=cit"re ,i9!"#-.Carea )e .iat!ia A


60/256

'ucia.a Do;


61/256


S&rsa e)o,e' )e .ita!ia D

*i.te>a e.(o-e.a a+ime.te

co+e*tero+

mucoa*a i.te*ti.a+a

(ehi(roco+e*tro+1

+umi.a

=O

co+eca+cifero+

;rin "ci!ne" r"di"ii#or UV 7+de)idroco#e(tero#!# for8"t


62/256

'ucia.a Do;a

hi(ro7ica+cifero+24

ca+cifero+!)24 ca+cifero+2")24ca+citrio+

ca+cifero+!)2")24

O=

O=

=O

ca+citrio+

62


63/256


Rol&l bioc$i!ic al .ita!iei D

Vit"8in" D "#-t!ri de )or8on!# "r"tiroidi"n inter,ine


64/256

'ucia.a Do;


65/256


/ita!ia E ;toco%eroliia

M-2

/ C=2C=O=C=2

=/C

=/C N

N N=

N

O

O

O P O P O C=2

O= O=

O OO

N

N

N

N

N=2

O==O

AD

Dintre "ce(te do!- for8e AD rere9int- 0 N > 20i"r ri&of#",in" #i&er- r-8ne


75/256


Rol bioc$i!ic

A8&e#e f#",in+n!c#eotide N ADB (!nt coen9i8e c"re

f"c "rte din (i(te8e en9i8"tice i8#ic"te


76/256

'ucia.a Do;


77/256


N

CON=2

O

O=O=

C=2 O P O P

O

O=

O

O

O=

C=2

O

N

N N

N

O=O R

N=2

R : NAD ;O$2 NAD;

?n ce#!#e#e "ni8"#e NADFe(te re9ent


78/256

'ucia.a Do;


79/256


/ita!ia B8

Vit"8in" /6 n!8it- i irido=in" ("! "der8in" ""re (!&

trei for8e &io#oic "cti,e intercon,erti&i#e. Vit"8in" /6(e -(ete


80/256

'ucia.a Do;a

ATP

ADPN

C=O

C=2=O

=/C

P

O

O=

O=

O=

O=P

O

=/C

=O

N

C=2

C=2N=2

PA'PO

PMP

;A*;O i ;; irido="8info(f"tB (!nt for8e#e "cti,e "#e ,it"8inei/6. E#e f!ncione"9- c" i coen9i8e


81/256


Deci


82/256

'ucia.a Do;;rotein AC;B. AC; f"ce "rte dintr+!n ((te8 8!#tien9i8"ti,i8#ic"t


83/256


o=id"re" "ci9i#or r"i dec"r&o=i#"re" o=id"ti,- " "#f"+ ceto"ci9i#order"d"re" "8ino"ci9i#or


84/256

'ucia.a Do;ima =CO/8

ATP CO2 ;ioti.8 e.>ima ADP a

*u;*trat

*u;*trat car;o7i+at

A(tfe# &iotin+en9i8"


85/256


Din !nct de ,edere (tr!ct!r"# "cid!# fo#ic conine


86/256

'ucia.a Do;a N

N

N

N

O=

=

C=2

=

=2N

N= Rfo+at re(ucta>

NADP=

=

NADP

N

N

N

N

O= =

C=2

=

=

=2N

=

N= R

D=F

T=F

T rere9int- 8et"&o#it!# "cti, i (er,ete dret coen9i8-


87/256


Ati%olice ; Ata,oi-ti %olici ,ite9" 8"=i8-re"#i9"t-Z S [ > concentr"i" de (!&(tr"tG8 > Con(t"nt" #!i ic)"e#i( > enten rere9int- concentr"i"de de (!&(tr"t entr! c"re ,ite9" de re"cie "tine '!8-t"te"

,"#orii 8"=i8e.

V

V8"=

V8%2

G8 Z S[

50


101/256


Do9-ri#e de "cti,it"te en9i8"tic- (e efect!e"9- #" concentr"ii8"ri ("t!r"nte de (!&(tr"t entr! " "(i!r" o cinetic- de ordin!#9ero.

Ec!"i" ic)"e#i(+enten e(te f!nd"8ent"#-


102/256

'ucia.a Do;


103/256


Un "#t ti de in)i&iie re,er(i&i#- e(te i$ibiia eco!*etiti.';ico!*etiti.'


104/256

'ucia.a Do; 600C 8"'orit"te" en9i8e#or (!nt in"cti,e. E=ceie f"c !ne#e8icroor"ni(8e ter8ofi#e "#e c-ror en9i8e (!nt "cti,e i #" 00C.

50


105/256


3. E%ect&l *H>&l&i as&*ra acti.it'ii e+i!atice.

En9i8e#e (!nt (t"&i#e


106/256

'ucia.a Do;ima

+ "("rt"t tr"n(c"r&"8i#"9" en9i8- ce c"t"#i9e"9- ri8" re"ciedin roce(!# de (inte9- " ri&on!c#eotide#or iri8idinice.

I!*ortaa bio!e)ical' -i %ar!ace&tic'

En9i8o#oi" "d!ce !n "ort i8ort"nt "tt


107/256


E=e8#!: #"ct"t de)idroen"9" cre"tin in"9"tr"n("8in"9e#e.4. E+i!ele i)&ctibile (inteti9"te in"9" re9int- $ i9oen9i8e i "n!8e: CG5: // CG2. / CG$:

Ace(te i9oen9i8e (!nt do9"te


108/256

'ucia.a Do;


109/256


/II( METABOLISM

et"&o#i(8!# rere9int- tot"#it"te" tr"n(for8-ri#or de

(!&(t"n- enerie i infor8"ie c"re "! #oc


110/256

'ucia.a Do;


111/256


/III( CICLUL ACIILOR TRICARBO4ILICI

Re(ir"i" e(te roce(!# rin c"re ce#!#" "ero&- o&ine enerie


112/256

'ucia.a Do;


113/256


?n et"" " do!" r!"re" )idro=ieti# e(te de)idroen"t- de

ir!,"t de)idroen"9- i"r r!"re" "ceti# c"re re9!#t- e(te tr"n(fer"t-#" "cid!# #ioic c"re rere9int- r!"re" ro(tetic- #e"t- co,"#ent #"ce" de " do!" en9i8- di)idro#ioi# tr"n("ci#"9-.

E5+ T;; + C + C$ F E2

O S S

E5+ T;; F E2

S S

O C + C$

Tr"n(fer!# !nei erec)i de "to8i de )idroen de #" r!"re")idro=i# " T;; #" #e-t!r" di(!#f!ric- " "cid!#!i #ioic f"ce c" "ce"(t"din !r8- (- tre"c-


114/256

'ucia.a Do;


115/256


2( O=id"re" i9ocitr"t!#!i #" -cetoglutarate(te o re"cie c"re"re #oc


116/256

'ucia.a Do;


117/256


!8"r"9" "cione"9- (tereo(ecific deo"rece e" for8e"9-n!8"i (tereoi9o8er!# * "# 8"#"t!#!i.

:( ?n !#ti8" re"cie " cic#!#!i *+8"#"t!# e(te o=id"t #"o="#i#"cet"t


118/256

'ucia.a Do;


119/256


Si(te8!# de en9i8e c"re de(er,ete #"n!# re(ir"tor e(te#oc"#i9"t


120/256

'ucia.a Do;CoA

CO2

citrat

i+ocitrat

CO2

>ceto,l&tarat

s&cciil>CoAs&cciat

%&!arat

!alat

o3alilacetat

CO2

52


121/256


I4( ENIME TRANSPORTOARE DE ELECTRONI

?n tr"n(ort!# de e#ectroni de #" (!&(tr"te or"nice #" o=ien!#red!c-tor "rtici- "tr! ti!ri de en9i8e:

5. de)idroen"9e #e"te de !n n!c#e! iridiniciridindeendenteB c"re nece(it- NAD ("! NAD; c" icoen9i8eL

2. de)idroen"9e #e"te de n!c#e! f#",inicf#",indeendenteB c"re conin AD ("! N c" ir!"re ro(tetic-L

$. roteine c! fier+(!#fL4. citocro8ii c"re conin ine#!# orfirinic c! fier c" i

r!"re ro(tetic-.?n #!( f"- de "ce(te roteine entr! tr"n(ort!# de e#ectroni 8"i

"rtici- i !&i^!inon" ("! coen9i8" X.De$i)ro,ea+e le,ate )e *iri)i'(

Deo"rece "ce(te en9i8e nece(it" NAD ("! NAD; dret coen9i8e"ce(te en9i8e (e n!8e(c de)idroen"9e iridin deendente. Sec!no(c 8"i &ine de do!-(!te de "(tfe# de en9i8e c"re f!ncione"9-


122/256

'ucia.a Do;


123/256


deci (i(te8!# NAD%NADFo"te tr"n(fer" e#ectroni de #" !n(!&(tr"t #" "#t!#


124/256

'ucia.a Do; (!#f de(coerit-feredo=in" " fo(t identific"t- (!#f "! fo(t -(ite i


125/256


NAD+de)idroen"9" do!- centre (!nt "(oci"te citocro8!#!i & i!n!# citocro8!#!i c5.

Citocro!ii

Citocro8ii (!nt roteine tr"n(orto"re de e#ectroni c"re conin


126/256

'ucia.a Do;


127/256


Calea tras*ort&l&i )e electroi ;la&l res*irator c"#e" ento9ofo(f"t!#!i 6 > c"#e" deintercon,er(ie #icoeno#i9".

5$


135/256


Ficat&l !ti#i9e"9- #!co9" "(tfe#:+ o "rte o !ti#i9e"9- entr! nece(it-i roriiL+ o "rte o deo9ite"9- (!& for8- de #icoenL

+ ce" 8"i 8"re "rte e(te re!(-


136/256

'ucia.a Do;


137/256


fo(fo0#icer"t

8!t"9"

C2O

+C+O;

COOeno#"9"

2O

COO

C+O

C2

;ir!,"t in"9"

AD; AT; C2

C+O

COO

C$

C O

COO#"ct"t de)idro0en"9"

NAD F F

NADF

COO

+ C + O

C$

I!*ortaa ,licoli+ei+ #ico#i9" e(te o et"- oi"torie


138/256

'ucia.a Do;


139/256


4(1( Calea *eto+o%os%atic'

O "#t- c"#e de der"d"re " #!co9ei


140/256

'ucia.a Do;


141/256


ri&!#o9o+3+fo(f"t

ri&o9o+3+fo(f"t

=i#!#o9o+3+fo(f

+C+O

+C+O

C2+O+;O

$

2

CO

C2+O ri&!

#o9o+

3+fo

(f"t

i9o

8er"9

"

+C+O+C+O

C2+O+;O$2

CO

+C+O

ri&!#o9o+3+fo(f"t

e7i8er"9"

O+C+

+C+O

C2+O+;O$2

CO

C2+O

Ace(te tr"n(for8-ri (!nt re,er(i&i#e i"r ec)i#i&r!# (e (t"&i#ete


142/256

'ucia.a Do;


143/256


f"t!# c- nece(it- ec)i"8ent en9i8"tic re#"ti, red!( de !nde iden!8ire" de \!nt ento9ofo(f"t].Patolo,ia c'ii *eto+o%os%at

#!co9o+6+fo(f"t de)idroen"9" e(te o en9i8- ce re9int-roriet"te" de o#i8orfi(8 enetic. Ace"(t"


144/256

'ucia.a Do;


145/256


O

C2O

OO

O

O + ; + O + ; + O + ; + O + 2C

O O O

O OO

O

O

O O

N

N

O

UD;+#!co9"

?n et"" !r8-to"re UD;+#!co9" (e o=ide"9-


146/256

'ucia.a Do;


147/256


co8!ii to=ici de oriine e=oen- ("! rod!i re9!#t"i


148/256

'ucia.a Do;


149/256


4(6( 5l&coeo,ee+a

;roce(e#e de c"t"&o#i9"re " #!cide#or


150/256

'ucia.a Do;


151/256


F T;C2

COOC O

COO

0F2C2

COO

C + O ;O$2 F D; F CO2

Ec!"i" #o&"#- " roce(!#!i e(te:

C$

C O F AT; F T;

COO

C2

COO

C + O ;O$2 F D; F AD; F ;"

Deci entr! fo(fori#"re" eno#ir!,"t!#!i #" fo(foeno#ir!,"t "fo(t nece("r- eneri" !nei 8o#ec!#e de AT; i !n" de T;. ?ncontin!"re fo(foeno#ir!,"t!# for8"t (e tr"n(for8- rin in,er("re"re"cii#or #ico#itice n- #" (t"di!# de fr!cti9o+56+difo(f"t.

Hi)roli+a %r&cto+o>0#8>)i%osa%at&l&ie(te " do!" re"cie dinc!r(!# &io(inte9ei #!co9ei c"re "re !n 8ec"ni(8 rori! i n! con(t-dintr+o in,er("re " re"ciei din c"dr!# #ico#i9ei.

r!cto9o+56+difo(f"t F 2O fr!cto9ofo(f"t"9" r!cto9o+6+fo(f"t F ;"

?n et"" !r8-to"re fr!cto9o+6+fo(f"t!# (!& "ci!ne"i9o8er"9ei e(te tr"n(for8"t-


152/256

'ucia.a Do;


153/256


4(7( Metabolis!&l ,lico,e&l&i

#icoen!# rere9int- for8" 8"'or- de (toc"re !or

di(oni&i#-B " e=ce(!#!i de #!co9-


154/256

'ucia.a Do;


155/256


#!co9o+5 fo(f"t. #!co9o+5 fo(f"t!# re"cione"9- c! UT; entr! "for8" UD;+#!co9". UD;+#!co9" re"cione"9- c! o 8o#ec!#-inii"#- de #icoen ree=i(tent- din 8et"&o#i(8!# #icoen!#!i

den!8it- inii"tor ri8erB e c"re ,or fi tr"n(fer"te !n" cte !n"!nit-i de #!co9- de e for8" "cti,"t- " "ce"(tei".*"


156/256

'ucia.a Do;


157/256


a)realia

DA di"ci#0#icerideB

C2OOC+R5

COOC + R2

C2O

I$;$ino9ito#+$+fo(f"tB

O O

O;

O

O

;O

Ce#e do!- c-i 8et"&o#ice #icoeno#i9" i iconeoene9"(!nt di(tincte d"r 8"i "#e( cnd (e rod!ce !n inf#!=de ioni de c"#ci! din e=terior ("! o"te fi o "ci!ne de 8"i #!n-d!r"t-


158/256

'ucia.a Do;


159/256


4(8( 5lico*roteie -i *roteo,licai(

Co&,aii ,l&ci)elor

S!nt co8#eci for8"i rin "(ociere de #!cide c! roteinei%("! #iide de e=e8#! #icoroteine#e roteo#ic"nii#ioroteine#e etc.

#icoroteine#e i roteo#ic"nii (!nt co8!i c"re (efor8e"9- rin "t""re" co,"#ent- de #"n!ri o#io ("! o#i9")"ridice #"(c)e#ete o#ietidice. Di(tinci" (e f"ce "c. N+"ceti#ne!r"8inicB+ f!co9"

O

C2O

O

N+CO+C$

O

O

OO

N+CO+C$

O

C2O

O

N+"ceti#0"#"cto9"8in" N+"ceti#0#!co9"8in

C2O

O+C+

O COO

O2N+C+

+C+O

+C+O

C2O

O

+C+O

+C+O

O

N2

O

OOC

C2O

2N+C+

+C+O

+C+O

COO

C+O

C2

C+

+C+O

O

"cid (i"#ic NANAB "ci#"re" "c. Ne!r"8inic

53


160/256

'ucia.a Do;


161/256


Eta*ele site+ei5. A("8"re" #"n!#!i o#io9")"ridic i tr"n(fer!# (-! e

(c)e#et!# etidic.

2. ;re#!cr"re" #icoroteinei for8"te P

Dol>P>P>5l&

UD;+0#!

U;

UD;+N+"ceti#+0"#

UD;

Dol>P>P>,l&>N>Ac>,al

D;+8"no9"

D;

Dol>P>P>,l&>N>Ac>,al>!a

Dol>P>P>oli,o+a$ari)a

#"nt 7o#i7e7tid

,lico*roteia *ri!ara

o#i0o9")"rid tr"n(fer"9"Dol>P>P

Dol>P

P

56


162/256

'ucia.a Do; #icoroteine#e ce tr"n(ort- ioni

8et"#ici #iide ,it"8ine etc.+ ro# #!&rifi"nt i rotecie > 8!cin"+ ro# en9i8"tic > f"ctori de co"!#"re+ !nii )or8oni (!nt #icoroteine

+ ro#


163/256


O

O

O

COO

O

OO

OC2O

C$CON

O

1( Co)roiti>s&l%aii (!nt #ico9"8ino#ic"nii 8"'ori dinroteo#ic"nii c"rti#"ii#or "rtere#or corneei fiind "#c-t!ii din "cid#!c!ronic i N+"ceti#"#"cto9"8in- #e"te P+5$+#!c!ronidic i P+54+"#"cto8idinic. N!8-r!# 8"re de ("rcini ne"ti,e din 8o#ec!#"condroitin(!#f"i#or


164/256

'ucia.a Do;


165/256


Sc)e8" 8et"&o#i9-rii "#"cto9ei

)&lcitol

NAD F F

NAD

F

"#dored!ct"9",alacto+a

)e=oin"9"

AT; AD;

,alacto+o>8>%os%at

AT;

AD;

0"#"ctoin"9" 5B

,alacto+o>0>%os%at UDP>,l&co+a

UDP>,alacto+a ,l&co+o>0>%os%at ,lico,e

,l&co+o>8>%os%at

,l&co+a 0#ico#i9"

;ri8" et"-


166/256

'ucia.a Do;a

UDPL-a+ UDPL -+u

e%imera>a

Ace(te re"cii "! #oc


167/256


"#+5; c"re e(te !n rod!( to=ic entr! or"ni(8. *" coii #i(""ce(tei en9i8e e(te de(t!# de (e,er- deo"rece UD;+"# n! (e 8"ifor8e"9-. S!"r!# re9int- t!#&!r-ri de cretere i"r


168/256

'ucia.a Do; tioin"9"G > #icero#in"9"

r!cto9" (e "d8ini(tre"9- di"&etici#or (!& for8- de erf!9ii.r!cto9" (e tr"n(for8-


169/256


Glu Sor F

NADH+H+ NAD+ NAD+

NADH+H+

AR Sor_DH_a!a

As*ecte )e *atolo,ie

Deficien" fr!ctoin"9ei d!ce #" ""rii" en9i8o"tiei n!8it-fr!cto9!rie e(eni"#- n! (e fo(fori#e"9- fr!cto9" "ce"(t" (e"c!8!#e"9-


170/256


171/256


+ for8e de deo9it"re i de tr"n(ort "# co8&!(ti&i#i#or+


172/256

'ucia.a Do;


173/256


C


174/256

'ucia.a Do;


175/256


4I(1( Metabolis!&l aci+ilor ,ra-i sat&rai

Catabolis!&l aci+ilor ,ra-i

Aci9ii r"i (e der"de"9- rin ced"re" (!cce(i,- " !norfr"8ente de 2 "to8i de C


176/256

'ucia.a Do;+is' ;> o3i)areaito+

;rin fo(fori#"re" r!-ri#or O din ino9ito#


189/256


car(io+i%i.a

fo*fati(i+-+icero+

fo*fati(i+-+icero+fo*fat

C=2 OCO R!

O=O=

O P O

O

C=2 C= C=2 O P O

O

C=2

C= OCO R2

C=2

R2 COO C=

R! COO C=2

O=

CMPCDPL(iaci+-+icero+

O=

R! COO C=2

C=R2 COO

C=2 O P OO

C=2 C= C=2 O=

O=

fo*fati(i+-+icero+fo*fata>a

=/PO"=2OO=

O=C=2 O P

O

O=

O P O

O

C=2

C= O=

C=2

R2 COO C=

R! COO C=2

CMP

O=

C=2 O P O=

OC= O=

O=C=2

O=O=

O P O

O

P citi(i+

OR2COO C=

C=2

C=2 OCO R!

1( Biosite+a ,licerol%os%oli*i)elor *lec") )e la 0#1>)iacil,licerol

;e "ce"(t- c"#e (e (inteti9e"9- #ecitin" i cef"#in"


190/256

'ucia.a Do;


191/256


N

N

N

N=2

O

O=

C=2 S C=/

O=

C=22

C= N=2

COO=

C=2 OCOR!

C= OCOR2

C=2 O P

O

O C=2 C=2 N=2

O=

/

/

COO=

C= N=2

C=22

O=

N

N

N

N=2

O

O=

C=2 S

O=

O C=2 C=2 NC=//P

O

OC=2

OCOR2C=

C=2 OCOR!

SAM

+eciti.a

SA=

Biosite+a %os%ati)ilseriei

?n e(!t!ri#e "ni8"#e (inte9" fo(f"tidi#(erinei con(t-


192/256

'ucia.a Do;a

A!

D

C

A2

o(fo#i"9" A5 (cinde"9- #e-t!r" e(ter din o9ii" 5 "fo(fo#iidei e#i&ernd "cid!# r"(. o(fo#i"9" A2 "cione"9- "(!r"#e-t!rii e(ter din o9ii" 2 e#i&ernd "cid!# r"( ne("t!r"t. ;rin


193/256


o(fo#i"9" A2c"re (cinde"9- "cid!# r"( ne("t!r"t din o9ii"2 f!rni9e"9- "(tfe# "ci9i r"i ne("t!r"i entr! (inte9"ro(t"#"ndine#or tro8&o="ni#or i #e!cotriene#or. ?n "ncre"tit"

"c!t- fo(fo#i"9" A2 trece


194/256

'ucia.a Do;i.a

A#te e=e8#e de (fino#iide: Sfino8ie#in" cere&ri9ide#e (!#f"tide#e i "n#io9ide#e.

De,ra)area s%i,oli*i)elor

Sfino#iide#e (!nt der"d"te de c-tre (fino8ie#in"9- oen9i8- c"re


195/256


4I(9( Metabolis!&l colesterol&l&i

Co#e(tero#!# e(te !n (tero# 8"'or


196/256

'ucia.a Do;)idro=iP+8eti#+

#!t"ri#+CoA+(intet"9ei +CoA+(intet"9"B i for8e"9- +CoA. "i de"rte r!"re" c"r&o=i#ic- #e"t- de CoA e(te red!(- #"r!"re )idro=i#ic- c! e#i8in"re de CoA. Re"ci" "re #oc a

=OOC C=2 C

O=

C=/

C=2 COSCoA

C

O=

C=/

C=2 COSCoA=OOC C=2 2NADP= 2=

=MLCoA re(ucta>a

=OOC C=2 C

O=

C=/

C=2 C=2 O= 2NADP

CoAS=

51


197/256


1( Tras%or!area aci)&l&i !e.aloic ? sc&aleAcid!# 8e,"#onic (e fo(fori#e"9-


198/256

'ucia.a Do;a

C

C=/

C==/CC=2

C=2

C

C=/

C=

C=2 O P O P O=

O O

O= O=

-era.i+%irofo*fat

O=O=

C=2 O P O P O=

O O

C=2=2CC

C=/

PP

C=2

C=

C=/

C=2C=2

C=/C

C

C=/

C=

far.e>i+%irofo*fat*i.teta>a

C=2C

C=/

C=C=2O P O P O=

O O

O=O=

far.e>i+%irofo*fat

O= O=

P O P O=

O O

C=C=2O

C

C=/

C=2

C

C=/

C==/CC=2

C=2

C

C=/

C=

C=22

CC=

C=2C=2

C

C=/

C=C=2

C=2

C

C=/

C=

C=2C=2

C=C

C=2C=2

C=C

C=2C=2

C=

C=/

CC=/

=/C

C=/

C=/ C=/

*cua+e.

2( Tras%or!area sc&ale&l&i ? colesterol

Sc!"#en!#


199/256


O

O2

*cua+e. 2)/Le%o7i(u+*cua+e.u+ui

=O

+a.o*tero+u+

*"no(tero#!# conine $ r!-ri 8eti# imo*tero+1L(ehi(ro(e*mo*tero+

51


200/256

'ucia.a Do;


201/256


C"ntit"te" de co#e(tero# e#i&er"t- de *D* deter8in- ,ite9"8et"&o#i(8!#!i co#e(tero#!#!i re#"re" #" ni,e# ce#!#"r "(tfe# e=ce(!#de co#e(tero# "c!8!#"t


202/256

'ucia.a Do;


203/256


"tero"8e. Ace(t f"t d!ce #"


204/256

'ucia.a Do;o7ico+ic

Din &i#- "ci9ii &i#i"ri (!nt de,er("i


205/256


die(ti" i "&(or&i" #or rec!8 i " ,it"8ine#or#io(o#!&i#e: A D E i G.

+ Aci9ii &i#i"ri "cti,e"9- #i"9" "ncre"tic- i

co#e(tero#e(ter"9" "ncre"tic-. De"(e8ene"


206/256

'ucia.a Do;


207/256


4I(:( Aci+ii ,ra-i eseiali ;A5Eceto"cid!#!i "n"#o"8ino"cid!#!i inii"# i " "cid!#!i *+#!t"8ic.

25


218/256

'ucia.a Do;a

=/C C COO=

=/C C= COO=

=OOC C=2 C=2 C= COO=

N=2

O

O

C COO==OOC C=2 C=2

N=2

+euci.Ltra.*ami.a>a

=/C C= C=2

C=/

COO=C

O

=/C C= C=2 C=

C=/

COO=

N=2


N=2

O

C COO==OOC C=2 C=2

tiro>i.Ltra.ami.a>a

=O C=2 C COO=

O

=OOC C=2 C=2 C COO=

O

C= COO=

N=2

=OOC C=2 C=2

N=2

C=2 C= COO==O

25


219/256


Re"cii#e de tr"n("8in"re (!nt !or re,er(i&i#e e#e (e otde(f-!r"


220/256

'ucia.a Do;


221/256


II.

%iri(o7a+Lfo*fatLLe.>ima

cetimi.a a+(imi.a

%iri(o7ami.Lfo*fatLLe.>ima

C= OE

N=2

COO=R2 C=

=2O

=2O

E

C=

N

COO=R2 C=

E

C=2

N

R2 C COO=

=2O

=2OE C=2 N=2

O

R2 C COO=

;irido="#+fo(f"t!# for8"t o"te (- reintre atra.*ami.a>a

N=/=

NAD=

=2ONADcetoaci( -+utamat

a+faLceto-+utaratami.oaci(

22


222/256

'ucia.a Do;


223/256


*+"8iono"cido=id"9" "re c" r!"re ro(tetic- N (trn(#e"t- de rotein" en9i8"tic-. Se -(ete


224/256

'ucia.a Do;a

CO2

C=2 N=2R

COO=

R C= N=2

;entr! c" re"ci" (- "i&- #oc i


225/256


E=e8#e de re"cii de dec"r&o=i#"re:

N

N

C=2 C= N=2

=

COO=

CO2 =

N

N

C=2 C= N=22

hi*ti(i.a hi*tami.a

=2C C=

O=

COO=

N=2 CO2

=2C C=2 N=2

O=

*eri.a co+ami.a

hi(ro7i+a>a

*eroto.i.a

=N

C=2 C=2 N=2=O

CO2

=O

N

C=2 C= COO=

N=2

=

O2

=N

C=2 C= COO=

N=2

Din dec"r&o=i#"re" )i(tidinei tritof"n!#!i 3+)idro=itritof"n!#!i tiro9inei re9!#t- )i(t"8in" trit"8in"

(erotonin" i tir"8in" c! ro# de )or8oni ti(!#"ri. Din dec"r&o=i#"re"treoninei ci(teinei i "cid!#!i "("rtic re9!#t- ro"no#"8in"ci(te"8in" P+"#"nin" c"re intr-


226/256

'ucia.a Do;


227/256


A8oni"c!# rod!( #" ni,e#!# rinic)i#or deri,- din "8ino"ci9iiintr"ce#!#"ri i a

N=2


N=/=2ON=2O

=2N C C=2 C=2 C= COO=

A8oni"c!# o"te fi e=cret"t (!& for8- de (-r!ri de "8oni!."re" 8"'orit"te e(te e=cret"t- (!& for8- de !ree c"re (e for8e"9-


228/256

'ucia.a Do;a

-+utami. *i.teta>a

aci( -+utamic=2O

M-LADPaci( -+utamicM-LATP

=2N C C=2

O

C=2 C= COO=

N=2

N="

4II(6( Cicl&l &reo,eetic

22


229/256


?n c!r(!# re"cii#or ce "#c-t!ie(c !n cic#! co8#et dintr+!n 8o#

de "8oni"c re9!#t- !n 8o# de !ree i (e con(!8- $ 8o#i de AT;.Re"cii#e (!cce(i,e (!nt c"t"#i9"te de 3 en9i8e.C"r&"8oi# (intet"9" re9ent- a

or.iti.a citru+i.a

=/PO"O

N=2C=2 N=

C=2

C=2

C= N=2

COO=

C

=O P O

O

C N=2

O= O

C=2 N=2

C=2

C=2C= N=2

COO=

?n contin!"re citr!#in"


230/256

'ucia.a Do;


231/256


ar-i.i.a or.iti.a uree

O

C N=2=2Nar-i.a>a

=2O

C=2 N=2

C=2

C=2

C= N=2

COO=

C=2 N=

C=2

C=2

C= N=2

COO=

C

N=2

N=

Ornitin" re9!#t"t- o"te (- rei" !n no! cic#! i"r !ree" for8"t-e(te e=cret"t- !rin"r.

Deci cic#!# !reoenetic e(te o c"#e 8et"&o#ic- fo"rteco(ti(ito"re entr! or"ni(8 deo"rece entr! fiec"re 8o#ec!#- de

!ree ce (e for8e"9- (e con(!8- $ #e-t!ri 8"croerice.C"ntit"te" de !ree e=cret"t- de !n "d!#t nor8"# rin !rin- e(tede 23+$0


232/256

'ucia.a Do;


233/256


Sc$e!a ,eeral' )e !etaboli+are a sc$elet&l&i )e ato!i )e C aia!ioaci+ilor

I+e) Met)Thr)Va+

+.) +u) =i*)Pro) Ar-

Tr) Phe

A*%) A*.

I+e) 'eu) Tr

Tr) 'eu) '*)Phe) Tr

A+a) +) C*)Ser) Thr

acetoaceti+8 CoAaci( %iruGic

aci( ma+ic

aci( o7a+i+acetic

aci( fumaric

aci( *ucci..ic

*ucci.i+8 CoA

aci( a+fa8 ceto-+utaric

aci( i>ocitric

aci( citric

aceti+8 CoA

De%ecte ? !etaboli+area a!ioaci+ilor

?n #e-t!r- c! 8et"&o#i(8!# "8inono"ci9i#or (!nt de(cri(e8"i 8!#te "feci!ni c"!9"te


234/256

'ucia.a Do;


235/256


4II(8( Biosite+a a!ioaci+ilor

Dintre "8ino"ci9ii c!no(c!i n!8"i !n n!8-r de 20 re9int-i8ort"n- entr! &io(inte9" roteine#or. Une#e for8e de ,i"t-#"nte &"cteriiB (!nt c""&i#e (- (inteti9e9e toi cei 20 de "8ino"ci9idin inter8edi"ri "8fi&o#ici. A#te#e inc#!(i, 8"8ifere#e i


236/256

'ucia.a Do;a

5l&ta!ia (e (inteti9e"9- rintr+o re"cie c"t"#i9"t- de c-tre#!t"8in(intet"9- #ecnd de #" "cid *+#!t"8ic. Re"ci" nece(it-AT; c" i donor de enerie.

COO=

C= N=2

FC=22

COO=

N="

ATP ADP PM-2

COO=

C= N=2

FC=22

CON=2

-+utami.*i.teta>a

As*ara,ia(e (inteti9e"9- rintr+o re"cie (i8i#"r- #ecndde #" "cid *+ "("rtic (!& "ci!ne" ""r"in(intet"9ei AT; trece


237/256


C=2 OPO/=2

C= O=

COO=

=2O P

C=2 O=

C=

COO=

O=NAD NAD= =

C=2 O=

C O

COO=

AA A

C=2 O=

C

COO=

O

NAD NAD= =

C=2 OPO/=2

C O

COO= AA A

C=2 OPO/=2

C= N=2

COO= =2O

C"#e" rin inter8edi!# deri,"i#or fo(fori#"i nece(it-


238/256

'ucia.a Do;


239/256


Ci(tein" (e (inteti9e"9- din *+(erin- i )o8oci(tein- c"rero,in c" inter8edi"ri a

II

I=O C=2 C= COO=

N=2

NADPNADP= =

=2;io%teri.a=";io%teri.aN=2

C=2 C= COO=

Co8#e=!# feni#"#"nin )idro=i#"9ei re9int- do!- "cti,it"idi(tincte:5 > red!cere" o=ien!#!i 8o#ec!#"r #" "- i " feni#"#"ninei #" tiro9in-

2 > red!cere" di)idro&ioterinei #" tetr")idro&ioterin- e (ocote"#"NAD;.*" fe# c" i ro#in" )idro=i#i9in" e(te re9ent-


240/256

'ucia.a Do;


241/256


Hi)roli+a aci+ilor &cleici

*"n!ri#e #!ni " "ci9i#or n!c#eici (!nt )idro#i9"te de c-tren!c#e"9e


242/256

'ucia.a Do;a(e>o7iri;o.uc+ea>a

fo*fo(ie*tera>e

.uc+eoti(a>afo*fomo.oe*tera>e

.uc+eo>i(fo*fori+a>e

Ca+ea%e.to>ofo*fatu+u

R4LP(R4LP

R!LP(R!LP

P

Ba>e %irimi(i.iceBa>e %uri.ice

Nuc+eo>i(e

P

=2O4@LNuc+eoti(e

=2O

O+i-o.uc+eoti(e Nuc+eoti(e

=2O

'a.turi %o+i.uc+eoti(ice

Catabolis!&l ,eeral al &cleoti)elor la ba+e *&riice -i*iri!i)iice

N!c#eotide#e (!nt )idro#i9"te (ecific de c-tre 3H+n!c#eo9ide ifo(f"t. E=i(t- o 8"re ,"riet"te de "(e8ene" en9i8e !ne#e (ecret"tede 8!co"(" inte(tin"#- c! ro#


243/256


n!c#eotid !rinic for8"t rin (inte9" de no,o i re9int- !n !nct de


244/256

'ucia.a Do;i(fo*fori+a>a

=2N

N

N N

N

O=

= =

N

N N

N

O

=2N

==N

N N

N

O

=O=

-ua.i>i.L4@Lfo*fat-ua.i>i.a

-ua.i.a

7a.ti.a

-ua.a>a

24


245/256


N

N N

N

O=

O

O=O=

=2CO/PO82

=2O P=2C

O=

O

O=

N

N N

N

O=

=O

fo*fomo.oe*tera>a

P RL!LP

%uri.L.uc+eo>i(fo*fori+a>a

N

N N

N

O=

= =

N

N N

N

O

=

i.o>i.L4@Lfo*fat i.o>i.a

hi%o7a.ti.a

=2OO2 =2O2

NAD

7a.ti.a

=O =

N

N N

N

O

=NAD=

=

?n or"ni(8!# !8"n ="ntino=id"9" (e -(ete 8"i "#e(


246/256

'ucia.a Do;


247/256


*" o8 (e e#i8in- rin !rin-


248/256

'ucia.a Do;a

AT P ADP P

M-2

=O P

O=

O

=2C

O=O=

ON=

OC

C=2 N=2

$. De e N3 N50>8eteni#+tetr")idrofo#"t o tr"n(fer"9- trecefo#o(ind o 8o#ec!#- de "- o r!- for8i# e "to8!# de "9ot ro,enitdin #icoco#. ?n "ce(t fe# (e co8#ete"9- "to8ii din cic#!# ent"to8ic"# !rinei.

OC

C=2 N=2

ON=

O=O=

=2CP

O=

O

=O tra.*fera>a

T=FN4N!0Lmeti+e.

T=F

=O P

O=

O

=2C

O=O=

ON=

OC

C=2 N=

C= O

4. o(fori&o9i# + N > for8i##icin" din et"" recedent- (etr"n(for8- rin "ci!ne" !nei (intet"9e c"re (c)i8&- CO " "8idei c!N ro,enit de #" #!t"8in-. Re"ci" nece(it- )idro#i9" AT;.;rod!(!# for8"t e(te fo(fori&o9i#+N+for8i# #icin"8idin-.

OC

C=2 N=

C= O

ON=

O=O=

=2CP

O=

O

=O+. +u

M-2

ATPADP

=2OP

=O P

O=

O

=2C

O=O=

ON=

C

C=2 N=

C= O=N

3. Uti#i9nd eneri" #i&er- e#i&er"t- rin )idro#i9" !n!i re(tfo(f"t din AT; o "#t- (intet"9-


249/256


for8e"9- 3H+fo(fori&o9i#+3+"8inoi8id"9o#. r!" N "d-!"t-


250/256

'ucia.a Do;aM.2

ADP PATP

=OOC C=2 C= COO=

N=2

=OOC

=2N C

C N=

C=

N=O P

O=

O

=2C

O=O=

O

N=

C=

=OOCC=2 COO=

. ;rod!(!# re"ciei "nterio"re e(te (cind"t de c-tre o #i"9- #" 3

>fo(fori&o9i#+4+c"r&o="8id+"8inoi8id"9o# i "cid f!8"ric. Re"cii#e7 i (e "(e"8-n- (inte9ei "rininei din citr!#in- din cic#!#!reoenetic.

=OOC C=2 COO=

=N

C=

CO

O

O= O=

=2CP

O=

O=O N

C

C N=

C==2N

aci( fumaric

a(e.i+at*ucci.at +ia>a

=2NC

C N=

C=

N=O P

O=

O

=2C

O=O=

O

CO=2N

1. ?n et"" !r8-to"re e(te tr"n(fer"t- o r!- for8i# de e N

50

for8i#tetr")idrofo#"t d!cnd #" 3+fo(fori&o9i#+4+c"r&o="8id+3+for8"8idoi8id"9o# c"re conine toi "to8ii cic#!#!i !rinic.

23


251/256


CO=2N

O

O= O=

=2CP

O=

O

=O N

C

C N=

C==2N

formi+tra.*fera>a

T=FN!0Lformi+T=F C

C N=

C=

N=2C

O=O=

O

CO=2N

OP

=NO=C

50. ?n fine a

LCO2

P OO

N

N

N

N

O=

O= O=

=2C

Se o"te o&(er," c- entr! (inte9" I; (e con(!8- "(e#e-t!ri 8"croerice con(idernd i for8"re" ;R;;.

As*ecte )e *atolo,ie )i !etabolis!&l &cleoti)elor

5&tae(te !n" din ce#e 8"i r-(ndite &o#i de 8et"&o#i(8 c!o frec,en- de 0$ din o!#"i" E!roei. S!& "ce(t- den!8ire (!ntinc#!(e !n r! )eteroen de &o#i c"r"cteri9"te to"te rin)ier!rice8ie c"re d!ce #" de!nere" !r"t!#!i de (odi!


252/256

'ucia.a Do;


253/256


4I/( Metabolis!&l $e!o,lobiei

Deo"rece e=i(t- o ierdere er8"nent- de )e8o#o&in- rinc"t"&o#i(8 i rin


254/256

'ucia.a Do;


255/256


"oi #" &i#i,erdin- c"re (!& "ci!ne" &i#i,erdin o=id"9ei i NAD; e(tetr"n(for8"t-


256/256

'ucia.a Do;

curs biofarmacie

Documents