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-glucose polymer -storage carb in animals -branched -alpha 1-4 straight -alpha 1-6 branches -branches every 8-10 residues -stores in liver, skeletal muscle, kidney |
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-conversion of free glucose to glucose-6-phosphate -enzyme-hexokinase or glucokinase -requires ATP -conversion of G-6-P to G-1-P -enzyme phosphoglucomutase |
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-synthesis of UDP-glucose -glucose needs to be activated to add it to glycogen -enzyme-UDP-glucose pyrophosphorylase -pyrophosphate (PPi) is product -broken down by pyrophosphatase |
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-synthesis of glycogen "primer" -if glycogen stores are not completely depleted , UDP-glucose can add to current glycogen molecules -if depleted use glycogenin as an acceptor for first UDP-glucose -UDP-glucose reacts with OH of Tyr residue on glycogenin -glycogenin auto catalyzes first few additions (1-4 linkages) |
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-elongation of glycogen chains -enzyme-glycogen synthase -transfer of glucose from UDP-glucose to the growing end of the chain (nonreducing end) -C1 of UDP-glucose and C4 of accepting residue -alpha 1-4 glycosidic bond |
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-formation of alpha 1-6 branches -enzyme-4:6 transferase -breaks every 6-8 glycosyl residues (alpha 1-4 bond) -reattaches it to a nonterminal residue by alpha 1-6 bond -both nonreducing ends can be elongated by glycogen synthase -increases number of nonreducing ends -can be synthesized and degraded faster |
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-not a reversal of synthesis -mobilizes stored glycogen quick |
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-shortening of glycogen chains -enzyme-glycogen phosphorylase -cleaves alpha 1-4 linkages until 4 residues from a branch (limit dextrin) -results in release of G-1-P |
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-removal of alpha 1-6 branches -enzyme-debranching enzyme (2 activites) -4:4 transferase-transfers 3 of 4 remaining glycosyl residues to another nonreducing end -1:6 glucosidase-hydrolytic cleavage of alpha 1-6 bond, releasing free glucose |
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-conversion of G-1-P to G-6-P (for use in cells) -enzyme-phosphoglucomutase -reverse of glycogenesis rxn -G-6-P to glucose -enzyme glucose-6-phospatase |
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-glycogenesis-active in liver during high BG -active in muscle during periods of rest |
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-glucagon-activates glycogenolysis -insulin-activates glycogenesis |
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-glycogenesis-glycogen synthase is activated by substrate G6P -glycogenolysis-glycogen phosphorylase is inhibited by G6P, glucose, and ATP |
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-glycogen storage diseases -Pompe,McArdle,Cori Diseasec -defects in enzymes, abnormal glycogen structure |
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-cleaves 1-4 links until 4 residues from each branch (limit dextrin) -releases G-1-P |
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-transfers 3 residues to another non reducing end |
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-hydrolyitic cleavage of 1-6 bond releasing glucose |
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