pyruvate dehydrogenase .

grooming phase
.0
CoA ,
ATP 4- ( NADH .)pyruvate dehydrogenase-
- CO2 ( NADH,
.2
.)FADH2
- ,
.3
ATP ( NADH
.)ATP
, , .
, , () (
) . .
.

, .
. .
- ( ) . .
- . - ,
,
.
( ).
, , . (
, ).

:
( , )TCA
"" - , . ""
.acetyl CoA -
, , .
- - - .A
, ,
.
.
2 . - ,
"" , , ,
( .)heme -
"" ( ) .
- .CO2

 ,
ATP . , acetyl CoA
NADH .FADH2 -
, NADH - FADH2 -
( ) .
ATP ADP - -.
,
NAD+ - ,FAD -
( , NAD+
, ).
, , .
NADH .FADH2 -
,oxaloacetate - , .
, . oxaloacetate
.
:
.0 ,NADH FADH2 GTP.
.2 .
( ) ( ).
- G
.
G - ( 7-0 )
.
:19
:0
: () CoA ,oxaloacetate - ( H2O ) -
.citrate ' :
.
.
.20citrate synthase :
CoA :
:
( -9Kcal /mol =G0 : , ).
: ,
. , ( PEP--) ,
( , , , , ).

:2
: ( ) OAA-
. , ,
- . ,
' - .
Aconitase :
:
19

, , .
- Synthase 20 ATP .

 : .
-0.5Kcal /mol =G0 : .
: .

:3
:- ( --) .-Keto-glutarate -
-- .
21
Isocitrate dehydrogenase :
NADH + H+ : .CO2 - . CO2 -
. '
( )NAD ,FAD , .ATP-
: .
.-5Kcal /mol =G0 : .
:.

:4
:- ( --) -KG - CoA-SH
CO2 . succinyl CoA -- -- CoA
- pyruvate - .acetyl CoA 4
. succinyl CoA - , .
.- ketoglutarate dehydrogenase complex : .
.CO2 ,NADH + H+ : - .
.NADH -
:
.-7Kcal /mol =G0 : .
: -- ,
( , ).

:0
: CoA .succinate - succinyl CoA - -
( .)8Kcal/mol 0 -
21

NADH dehydrogenases
( - ).

 4 9 . ,
GDP 22GTP .
Succinyl - CoA synthetase :
.GTP : .
: .
( -0.8Kcal /mol =G0 : ).
: succinyl CoA - ( )heme.

GTP -' GDP -

.GTP ,histidine succinyl CoA .
CoA- , ,succinate GDP.GTP-
:
.0 23 .
.2 - 24 .

:9
: . FAD - NAD - .
FAD .NAD+
.
.Succinate dehydrogenase complex : .
.FADH2 : FADH2 - (
) 2 .
: .
0Kcal /mol =G0 :
:.

22

GTP :ATP . ,
, . . -
. . GTP - ATP .
23 , .Succinyl -
24
,.

 :7
: .
L .
Fumarase :
:.
:
-0.9Kcal /mol =G0 :
:

( 0 ):
: OAA - .NADH+H
( Malate dehydrogenase : )
NADH : H+
: .
.7Kcal /mol =G0 : :
)-9Kcal /mol =G0( 25 (
). ,
" - ,.
+

. :
.0 4 ,oxaloacetate , 2 9 -
.citrate ,
.2 citrate - .isocitrate -
.3 isocitrate - - , 0 .-keto-glutarate
.4 -keto-glutarate - - , 4 .succinyl CoA
Succinyl CoA .0 ' ,succinate .CoA-SH
.9 succinate - .fumarate-
.7 fumarate - ( malate - .)L
.0 malate - - .oxaloacetate -
:
.0 CO2
- . ,
.
.2 NAD+
- , FAD
,succinate NAD+ () .malate
.3 , .
.4 ( )GTP - -
succinyl CoA .succinate -

25

. , .

 , :
,CoA 2 .CO2
( 9 ,) Nicotinamide adenine ( NAD+
)dinucleotide
( 2) .)Flavic adenine dinucleotide( FAD
GTP .
:

:
( oxaloacetate- ,0 ) ,
. ,
( PEP--) , (,
, , , ).
( citrate- .)0
glutamate ()purines
( alpha-ketoglutarate- .)3
, heme ( succinyl CoA- .)4

:
. ATP - .
ATP , - . ADP , .
:
- Isocitrate dehydrogenase ,ADP
. - Mg2+ ,NAD+ , ADP . NADH ,
.NAD+ ATP . -
NAD+ ,FAD - .
- -keto-glutrate dehydrogenase
- . . succinyl CoA

 ,NADH ( ) . ,
. , .ATP
:
acetyl CoA .
ATP citrate synthase
CoA- ,
.
malate dehydrogenase " .NADH

- :
( ) .
. , 4,0,9
.
:
citrate
.0
- ketoglutarate . , .
.2
Oxaloacetate, Malate .
.3
,
. ,
( ) , ATP -
. , ,
("= Anaplerotic") -
( ) . :
aspartate( oxaloacetate- ); glutamate--
( ketoglutarate - ,)4 ( succinyl CoA- )0
( fumarate- .)7

?
. .
.
. ".
. , .
. , -.
. .

 :
:
3 .0 ( NADH 3 - ATP ).
( GTP .2 ATP).
( FADH2 .3 2 - .)ATP
02 . -
, " 24 .
:
.0 2 ATP 2 - ( NADH" 2*3 + 2 - = ATP
.)ATP 0
.2 PDH - 2 ( NADH .)ATP 9 -
24 - - ~ .32 ATP
:

>-

2
ATP 2
2
NADH

1 ATP
3 ATP

"
0
3 ATP
NADH Pyruvate
dehydrogenase
( >- 2X )CoA
" 2X pyruvate dehydrogenase-
0
1 ATP
( 2X GTP
)
0 FADH2
2 ATP
3
3 ATP
NADH
" 2X
"

"
2 ATP
6 ATP
6-8 ATP
3 ATP
6 ATP
1 ATP
2 ATP
9 ATP
24 ATP
36-38 ATP

, 011- 21- .ATP 011 ,
( 09) 01- .ATP
- ( ) .
( (16C ~ .129ATP

.
- , ,
. .
:
, , , .
, , . IQ 20 . .
, . , .
.

.
:
, -
. - , ,
- .

 .
. ,
.
' , ,NADH -
. (
).

, .
:
.
( ).
. , ATP
,synthase , ADP - .ATP -
( , ) .
.
02 . .
,
.ATP - .
( ).
:


( , , )
. p-
,side n-side - .
.

:
, , :
- NADH dehydrogenase .0 NADH -
.
- Succinate dehydrogenase .2 . , .
.Co-enzyme Q -

 .a , ,Q
, ().
.b ,cytochrome C - .
- Cytochrome reductase .3 .
- Cytochrome oxidase .4 - .
.

:
001- , 43 - . ,FMN Fe-s -
(-) . FMN - .FAD -
041- , 4 -.
Cytochrome reductase 231.
091 .
, ,
.

(:NADH dehydrogenase )0
NADH - , FMN : ,
- ,Q .01Q - ,
. 4 . 4 .
. FMN - .
- .

(( Succinate dehydrogenase )2 ):
FADH2 - - .Q FADH2 -
.
, ,FAD - . .Q
- ,Q , . Heme b
, .
- .Heme b -
, , .
succinate dehydrogenase - fatty acetyl CoA - .

:Glycerol 3-phosphate dehydrogenase shuttle

.
. .
, , (-3- -
, -3- , - ) .-
, . -
NADH -3- .
( - ) .

 , , ,succinate dehydrogenase -
.FAD , NADH ,FADH2 -
, .Q ,
ATP.
,
, .
,ATP "" ,
( NADH - )
, .
39-30 ATP .

:
NADH - , . -
NADH - . ,
,malate aspartate suttle( Malate-aspartate , ) ,
. .
, ,
. , .malate - ,
, , ,
. ( . , , -
)

 :)Ubiquinone( Q
.
,
. ,
Q - : .Q - Q 01 ( .)Q10
.
( , ,semiquinone radicle - )III
.

(:)Ubiquinol Cytochrome C reductase( Cytochrome reductase )3

Q . Q - .Fe-S C
, , Q -
. . Q
. Q - .
, Q - . Q -
. 4
( 0 2 - )3 ubiquinone( Q10 ) .
.
, .
. ,C cytochrome
.oxidase

:Cytochrome C
, . , .
C - .
(:cytochrome oxidase )4
. ,C
2 4 - . , 4 ( )N-side ( p-side -
-) ." 0 .
.ATP .

:
NADH . .
, ,NADH dehydrogenase - Q
. C , .
.
.succinate dehydrogenase FADH2 - .
. FADH2 .
ATP .
, NADH ,FADH2 .FADH2 -
. NADH
, . .ATP
. 91% .

 :
.ATP -
( ) . ATP .ADP

,ATP synthase , :
, F0
+.
)FoF1-ATPase( ATP synthase :
- Loose + .ADP + P -
+.
- Tight ADP + P - ( ATP .)tight -
- Open .ATP -
ATP .-
ATP ,ATP synthase - ,ADP
ADP - . ADP - ATP - .

:
,)2 NADH + ATP 2( 6-8 ATP - NADH FAD - .ATP 2
( NADH 2 - CoA )
)GTP ,FADH2 ,NADH 3( 12*2 -
36-32 -' ATP .
:
, , ,
. . (
):

 - ( .)CO - .
NADH - .Q - . ,
.
( - ) - ,DNP - , ATP -
synthase ( ) . DNP -
, .
- ATP synthase , .ATP synthase
( ' ).

:
' , ,
, .
:
ATP - H -
.
, , ATP
. .
, .ATP ATP ,
+

- P/O :
ATP - ' .
ATP ,NADH - P/O - 3 ATP 3 '
- ' .ATP 3 ,ATP 2 - .0 ATP -
.ATP

:
:
. .
. pH - .
. .ATP synthase
. pH - .ATP synthase
:
. .
. .
. .
. .
. bilayer -( . C !)
():
http://www.youtube.com/watch?v=xbJ0nbzt5Kw

:ATP synthase -
http://www.sigmaaldrich.com/life-science/metabolomics/learning-center/metabolic-pathways/atp-synthase/atpanimation.html

:- - ( ,
" ").
:
:
. , .
21 : 4:



R
().
71-61 . ,
() ,
, .

71-61 , :
-
- .
- .
(- )transporters
- .
- . .
- "".
- .ubiquitin
. ,
.
, - .


.
- ( , , , )
- ( , , - cachexia ,
- )
:
.
.
.
:

. .
.
" " .
.

:
.0 ( pH - ).
.2 , , .
. :
.0 .
.2 ( ).
.3 .
, , .

:
21 R - , ,
( ) . 6 . ,
00 .
. .
. :

. :

**

**

, , .
.
, , .
. .
,
, .
.
, -
.

:
:
- - CoA
( CoA .)Keto
- .
. .
- , -.

:


Acetoacetyl CoA
Acetyl CoA
:

 .0 -3 - , ( )PEP.
.2 -- --.
.3 ,ribose-5-phosphate( petose phosphate pathway - eythrose-4-phosphate - -
PEP ).
(" ") .
.

" " .
.
-.
.
:
,.
-- , . ,
R - - .
.
, -- ,
. , .
0 , ,
.
- 4.
( ,
).
( --).
( ):
--

.