《医学生化》PPT课件

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Harper’s illustrated biochemistry 26th edition
Charpter 18
Metabolism of Glycogen
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1.Introduction and biomedical importance
2.Glycogen biosynthesis(glycogenesis)
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(4) Hydrolysis of the 1→6 linkages requires the debranching enzyme forms free glucose.
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糖原分解与合成的主要步骤及限速酶
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4. Regulation of glycogen metabolism
carbohydrate in animals, corresponding to starch in plants. It is a branched polymer of α–D-glucose. It occurs mainly in liver and muscle
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(2) Metabolism of Glycogen contains
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Biosynthesis of glycogen
glycogen primer
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3.Glycogenolysis
Glycogenolysis is not the reverse of glycogenesis but is a separate pathway.
(1) Glycogen phosphorylase catalyzes yield glucose 1-phosphate.
It is effected by a balance glycogen synthase and phosphorylase.
Glycogen synthase and phosphorylase activity are regulated by allosteric mechanisms and covalent modifications.
Cyclic AMP integrates the regulation of glycogenolysis and glycogenesis
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2.Glycogen biosynthesis (glycogenesis)
1. 概念 2. 合成部位 3. 过程
(1)
肝、肌肉和肾
hexokinase
glucose
glucokinase
glucose 6-phosphate,
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2.Glycogen biosynthesis (glycogenesis)
UDPGlc pyrophosphorylase UDPG“活性的葡萄糖”
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UDPG“活性的葡萄糖”
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4: Glycogen synthase catalyzes the formation of a glycoside bond.
5: Branching involves detachment of existing glycogen chains.
Glycogenesis and Glycogenolysis
Glycogenesis occurs mainly in muscle and liver.
Glycogenolysis is not the reverse of glycogenesis but is a separate pathway.
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Glycogen storage diseases are a group of inherited disorders characterized by deficient mobilization of glycogen or deposition of abnormal forms of glycogen, leading to muscular weakness or even death.
2.
phosphoglucomutase
Glucose 6-phosphate
Glucose 1-phosphate
3: Synthesis a special nucleotide of glucose (uridine diphosphate glucose, UDPGlc).
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酶:尿苷二磷酸葡萄糖焦磷酸化酶
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(2) glucose 6-phosphate can be formed from glucose 1-phosphate.
glucose 1- Glucose-6- phosphoglucomutase
phosphate.
phosphate
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(3)In liver and kidney 6-phosphateglucose
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biomedical importance
* Muscle glycogen is a readily available source of glucose for glycolysis within the muscle itself.
* Liver glycogen functions to store and export glucose to maintain blood glucose between meals. It can be used 8-12 hours after fasting.
glucose
glucose-6-phosphatase
glucose that is exported, leading to an increase in the blood glucose concentration.
In muscle, there is NOT glucose-6-phosphatase
3.Glycogenolysis
4.Cyclic AMP integrates the regulation of glycogenolysis and glycogenesis
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Introduction
(1).What is Glycogen? Glycogen is the major storage
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