GLYCOGEN SLIDES 2007-2008

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Transcript of GLYCOGEN SLIDES 2007-2008

2007-20081

2

3

6-

4

1-

5

-1,6 -1,6

6

-1,6

7

8

6-

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(PLP)

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11

:

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b, R T

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6-P ( )

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( )

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( Ca 2+) 4 (1200 Kd)

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G : GTP GDP : cAMP ATP PKA ( ) PP1 PP1 (a b)

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(UDP-)

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UDP- UDP-

2 Pi 21

(UDP-)

UDP22

( )1. 2. 3. . 8 UDP- ()

4.

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Structure of the glycogen particle. Starting at a central glycogenin molecule, glycogen chains (12 to 14 residues) extend in tiers. Inner chains have two (1n6) branches each. Chains in the outer tier are unbranched. There are 12 tiers in a mature glycogen particle (only 5 are shown here), consisting of about 55,000 glucose residues in a molecule of about 21 nm diameter and Mr 107.

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() : ( 7 > 11 ). - :

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97%.1 ATP 1 10% ( ) 1- . 31 .

6- 0 ATP 31 ATP CO2 + H2O

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1 (PP1)( PP1 )

PKA P P

PP1)

PP1

RGI RGI ( PP1 )

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1 (PP1)

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1 (PP1) () ()

PKA30

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( )

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: 1 (PP1)

P P

P

P

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()

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Glycogen-Engorged Lysosome. This electron micrograph shows skeletal muscle from an infant with type II glycogen-storage disease (Pompe disease). The lysosomes are filled with disease glycogen because of a deficiency in a-1,4-glucosidase, a hydrolytic enzyme confined to lysosomes. The amount of glycogen in the cytosol is normal.

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()

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