BIOXHMEIA, TOMO I ANEITHMIAKE EKOEI KPHTH

( ) (Gn)

Gn

()
> >

( )

()
> >

(G)
 G
H2O
( )

, -1,4 (..)

10 G -1,6

, G ,

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(10%
2% )

_1_ 1-PG
_2_
_3_ 1-PG 6-PG

G
UDP-G
( )

.
(

)
 4 :
-1,4 ..
() Gn

1-P ( ) ,
( ) 6-P ( )
==>
(C-O-C) (C-O-P),
Pi /(1-P-G) =100
 4
G
3 G

:

.

-1,6
 1--G 6--G

6--G

( . )

6--G ( )
6-- + H2O + Pi

( G
). 6--G ( G).
G . 4 ()


a b

BIOXHMEIA, TOMO I ANEITHMIAKE EKOEI KPHTH
 b

.
a

( - )
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( )

( ,
,
)




AMP, ATP



( T)
(R )

6-P-G
()

b ATP 6-P-G
() ATP 6-P-G AMP
b () AMP b
() ()
G

(90% ) a ()
() (
AMP ).
( )

;
G
,
. ;

(PKA)
AMP (cAMP)
Ca+ ( PKA)
 & ( )
()
( Tyr).
()

Tyr


( )


(
)

GTP
GDP

cAMP AMP



1

1



(GTPGDP & cAMP AMP)
/

29 NH2-His-Ser-Gln-Gly-Thr-Phe-Thr-Ser-Asp-Tyr-Ser-Lys-Tyr-Leu-Asp-Ser- Arg-Arg-Ala-
-Gln-Asp-Phe-Val-Gln-Trp-Leu- Met-Asn-Thr-COOH.

x 1 ( ) x 1000
cAMP

... ..

,


UDP-G

PPi

(
4 )



 -1,6

()

 (
PO4-2 ) ( b).

( )

( )


90% 1-P-G
( ATP)
10% G
ATP

31 ATP
1 ATP 6-P-G

97%



1 (PP1)

PP1
1) b a
2) ( a )

PP1 : PP1 , Rgi 1

PP1 RGi .
1 PP1
 : PKA
: PP1

H PP1
RGi
PP1
( H PP1 (
/ ) . )
RGi 1
PP1 PP1

 PP1

,

PP1
RGi

 (?) G (80-120mg/100ml 4,4-6,7mM)

a ( )

a ( ) .

G ,
G

.




a . G
R () T ( ).
T 14 PPI

T PPI
()

,
-

Von Gierke , ,
G .
6--G (6-- + H2O + Pi)
. ( ),
, .

Pompe ( )

. .

-1,6

 McArdle ( V)

, .

( G)

ATP
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