GLYCOGEN METABOLISM -...
Transcript of GLYCOGEN METABOLISM -...
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GLYCOGEN METABOLISM
DR. MD. MAHBUBUR RAHMAN
MBBS, M. Phil, MSc. (Biotechnology)
ASSISTANT PROFESSOR
DEPT. OF BIOCHEMISTRY
RAJSHAHI MEDICAL COLLEGE
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After the end of the session student will able to know the
• Biomedical importance of glycogen
• Definition of glycogenosis and glycogenolysis and flow chart
• Glycogen storage disease
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Glycogen
• Glycogen, the storage form of glucose, is a branched polysaccharide composed of chains of glucosyl units linked by α- 1-4 bonds with α- 1-6 branches every 8-10 residues.
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BIOMEDICAL IMPORTANCE
• Muscle glycogen provides a readily available source of glucose for glycolysis within the muscle itself.
• Liver glycogen functions to store and export glucose to maintain blood glucose level between meal .
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% of tissue
wt
Tissue
weight
Body
content
Liver
glycogen
5.0 1.8 kg 90 gm
Muscle
glycogen
0.7 35 kg 245 gm
Extracellular
glucose
0.1 10 L 10 gm
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Characteristics of enzyme related glycogen metabolism
• Glycogen synthase is a enzyme which is activated after dephosphorylation and inactive after phosphorylation.
• Glycogen phosphorylase is activated after phosphorylation and inactivated after dephosphorylation.
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Glycogenesis
a) Synthesis of UDP- Glucose
b) Synthesis of primer to initiate glycogen synthesis
c) Elongation of glycogen chain by glycogen synthatase
d) Formation of branches in glycogen
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Glycogenolysis
• Shortening of chains
• Removal of Branches
• Conversion of glucose 1 phosphate to glucose -6 phosphate.
• Lysosomal degradation of glycogen
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Regulation of glycogen synthesis and degradation
• Glycogen synthesis and degradation is accomplished on two levels.
– Glycogen synthatase and glycogen phosphorylase is allosterically controlled.
– The pathway of glycogen synthesis and degradation are hormonally controlled.
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Glycogen storage disease
• There are a group of genetic disease that result from a defect in an enzyme required for glycogen synthesis or degradation.
• They result either in formation of glycogen that has an abnormal structure or in the accumulation of excessive amount of normal glycogen in specific tissue as a result of impaired degradation
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continuation
Example
McArdele syndrom
pompe disease
Cori disease
Von gierke disease