: , , , , , ,

-1
. ,G-1-P
phosphoglucomutase ,G-6-P - 3 - :
.1 - .
.2 -.
.3 NADPH.
:
.1 -
. .
'.
.2 ) ( ,
.
, ,10%
2% , , " .
" ,"
.
" .

:
.1 .
.2 .
.3 .
.4 .
.5 .
.6.

-2
) UDP-glucose ( , G-1-P --
) UTP (uridine triphosphate" .UDP-glucose-pyrophosphorylase
UDP-glucose )(PPi
. " .
) ( --1,4
4 , 1 -UDP -
, .UDP 4 .
 -3 branching enzyme -
. ,
)-UDP -( .
. ,
, 7 . ,
.
" ) ,(branching enzyme 7 -
" --1,4 , --1,6 .
4 ,
11 . 8-12 =.10% -
,
= / .

-4
:
.1 ) ( , ,G-1-P
" .
Pi G-1-P -.
. .
.2 --1,6 - ) (debranching enzyme
.
" , ) "
(.
.3 G-1-P.G-6-P -
)' C-6 - ,
G-1,6-BISP - ' C-1
, (G-6-P
-6- , ER -,
G-6-P ,Pi - " .

) - 4-5
, ,
.
, PLP
) ,(pyridoxal phosphate )
,(4 .PLP -
, - . PLP - Pi - 1
(G-1-P
 -5
:
- ,a , ,b
.
2 R (relaxed) - ,-
) T (tense . a ,R b
.T -
a b - " )
(.
R T - . T
, R 10 ,
' .

:
= - .
= -
. .

-6 -
R T - ,b ) (R
AMP , , .R
ATP ,AMP T .
G-6-P .T
b .T
) AMP ,(ATP R ,b
. )( , ,
,a .

R T - ,a ,b - .
T a " ,
) (.
, AMP.G-6-P -

,
90% -.
 -7 -
, , " .
" .
4 : , -
, . - " .PKA ) ( ,
SR - .ER -
" , ,
a.b -

-8 )(:
.1 7TM .
.Gs
.2 Gs GDP " ,GTP -
) -(
. cAMP.ATP -
cAMP .3 ) ,PKA"
( . " .cAMP
PKA .4 ,
.
.5 PKA ,
. , , ) a( , ) b(
, .G-6-P

:
.1 G GTPase , GTP - -
,GDP .
.2 , cAMP.AMP -
,PKA .3 ,
, .
" ) ,PP1 (protein phosphatase 1 .
 -9 -
PP1 ,PKA - .
) Rg1 -( ) I -(.
Rg1 , ,PP1
) , (.
,Rg1 ,PP1
.
I ,PP1 - PP1 .

-10 -
,
= . )
( . , Rg1
,PKA ,PP1 - .
, ) ,(a
, .

, PP1 - .
)( . ,
T, PP1 , ,b
.

-11
:Von-Gierke disease -6- . G-6-P )
,(ER - . G-6-P
, .
.
G-6-P ,ER -
.

:Pompe disease -1,4-glucosidase -.

:Cori disease .debranching enzyme -
, .
:McArdle disease ,
.
 :
.1 -
.1 .branching enzyme - -1-
, 1-4
. 1-6
.2 ,1:10 branching enzyme -
8-12 ) 10(.
.2 --
.1 -6- ) (
.
.2 )
( . -6- -6-
)-6- b
. ( .-6-
.
.3 , -6-
.
.3 -
.1 )- ( ,
,
. , ,
.
ATP .2 ,
. , b
.ATP
.3 cAMP )
( , ) ( , - I
) (inhibitor ) PP1 ( . ,
cAMP - .
.4 AMP ,
. AMP - .
.4 :A
.1 - , .
.
.2 - ,
. .
.3 , .
 .4 - PP1 PP1. PP1 -
.
.
.5 - PP1 .PP1
.
.5 -
.1 .
.i - ,
, .
AMP .ii
.
. .iii
- .
:Pi .2
.i , ,
,ATP .
, .ii
.
.3 -6- ,
.
:PP1 .4
.i -
- .
- a .ii
- .