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[object Object],GLUCONEOGENESIS
GLUCONEOGENESIS ,[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
NONCARBOHYDRATE SOURCES ,[object Object],[object Object],[object Object],[object Object]
IRREVERSIBLE STEPS of GLYCOLYSIS ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
NEW STEPS in GLUCOSE FORMATION from PYRUVATE via GLUCONEOGENESIS ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Pyruvate carboxylase PEP carboxykinase Fructose-1,6-bisphosphatase Glucose-6-phosphatase
RECIPROCAL REGULATION OF GLYCOLYSIS & GLUCONEOGENESIS Glucose Fructose-6-phosphate Fructose-1,6-bisphosphate PEP Pyruvate Oxaloacetate PFK  F-1,6-BPase Several steps PK PEP  carboxykinase Pyruvate carboxylase GLUCONEOGENESIS F-2,6-BP  + AMP  + ATP  - Citrate  - H +   - F-2,6-BP  - AMP  - Citrate  + F-1,6-BP  + ATP  - Alanine  - AcetylCoA  + ADP  - ADP  -
GLYCOGEN  ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object]
GLYCOGENOLYSIS ,[object Object],[object Object],[object Object],[object Object]
Fates of Glucose-6-Phosphate ,[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Muscle,Brain Liver Glycogen phosphorylase Glucose-6-phosphatase Phosphoglucomutase
GLYCOGENESIS ,[object Object],[object Object],[object Object],[object Object],[object Object]
GLYCOGEN BREAKDOWN & SYNTHESIS ARE RECIPROCALLY REGULATED ,[object Object],Epinephrine Adenylate cyclase   Adenylate cyclase ATP   cAMP Protein kinase A   Protein kinase A Phosphorylase kinase   Phosphorylase kinase Phosphorylase b   Phosphorylase a Glycogen synthase a   Glycogen synthase b PINK – inactive   GREEN - active
GLYCOGEN STORAGE DISEASE TYPE DEFECTIVE ENZYME ORGAN AFFECTED GLYCOGEN IN AFFECTED ORGAN CLINICAL FEATURES I (Von Gierke) Glucose-6-phosphatase Liver & kidney Increased amount; normal structure Hepatomegaly, failure to thrive, hypoglycemia, ketosis, hyperuricemia, hyperlipidemia II (Pompe dse) α -1,4 glucosidase All organs Massive increase in amount; normal structure Cardiorespiratory failure causes death usually before age 2 III (Cori dse) Amylo-1,6-glucosidase (debranching) Muscle & liver Increased amount; short outer branches Like type 1 but milder IV (Andersen dse) Branching enzyme ( α -1,4 & 1,6) Liver & spleen Normal amount; very long outer branches Progressive cirrhosis of the liver; liver failure causes death before age 2 V (McArdle dse) Phosphorylase muscle Moderately increased amount; normal structure Limited ability to perform strenuous exercise because of painful muscle cramps. Otherwise patient is normal or well-developed. VI (Hers dse) Phosphorylase liver Increased amount Like type 1 but milder VII Phosphofructokinase muscle Increased amount; normal structure Like type V VIII Phosphorylase kinase liver Increased amount; normal structure Mild liver enlargement. Mild hypoglycemia
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Glycogen Metabolism

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  • 8. RECIPROCAL REGULATION OF GLYCOLYSIS & GLUCONEOGENESIS Glucose Fructose-6-phosphate Fructose-1,6-bisphosphate PEP Pyruvate Oxaloacetate PFK F-1,6-BPase Several steps PK PEP carboxykinase Pyruvate carboxylase GLUCONEOGENESIS F-2,6-BP + AMP + ATP - Citrate - H + - F-2,6-BP - AMP - Citrate + F-1,6-BP + ATP - Alanine - AcetylCoA + ADP - ADP -
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  • 16. GLYCOGEN STORAGE DISEASE TYPE DEFECTIVE ENZYME ORGAN AFFECTED GLYCOGEN IN AFFECTED ORGAN CLINICAL FEATURES I (Von Gierke) Glucose-6-phosphatase Liver & kidney Increased amount; normal structure Hepatomegaly, failure to thrive, hypoglycemia, ketosis, hyperuricemia, hyperlipidemia II (Pompe dse) α -1,4 glucosidase All organs Massive increase in amount; normal structure Cardiorespiratory failure causes death usually before age 2 III (Cori dse) Amylo-1,6-glucosidase (debranching) Muscle & liver Increased amount; short outer branches Like type 1 but milder IV (Andersen dse) Branching enzyme ( α -1,4 & 1,6) Liver & spleen Normal amount; very long outer branches Progressive cirrhosis of the liver; liver failure causes death before age 2 V (McArdle dse) Phosphorylase muscle Moderately increased amount; normal structure Limited ability to perform strenuous exercise because of painful muscle cramps. Otherwise patient is normal or well-developed. VI (Hers dse) Phosphorylase liver Increased amount Like type 1 but milder VII Phosphofructokinase muscle Increased amount; normal structure Like type V VIII Phosphorylase kinase liver Increased amount; normal structure Mild liver enlargement. Mild hypoglycemia
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