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GLYCOGENOLYSIS, Irreversible, Enzyme, Substrate, Product, rate limiting,…

- - - - Debranching enzyme
        
        Free glucose
        
        1 --> 6
        
        4-a-D-glucanotransferase
        
        transfers 3 residues to main branch, expose bond
        
        a-1,6 glucosidase
        
        Catalyzes hydrolysis of bond
  - - - Phosphoglucomutase
        
        G-6-P
  - - - Glycogen phosphorylase
        
        G-1-P
        
        1 --> 4
  - - - Glucose-6-Phosphatase
        
        Glucose
- - - - Active: dephosphorylated
      - Inactive: phosphorylated
    - - Active: phosphorylated
      - Inactive: dephosphorylated
  - - - Released from alpha cells of pancreas
    - - Released from adrenal medulla
    - - Adenylate cyclase activation
        
        ↑ cAMP = Activates PKA
        
        Phosphorylation of phosphorylase kinase
  - - - Inactive PKA cannot phosphorylate phosphorylase kinase = stimulates glycogenesis
        
        Activates glycogen synthase
        
        Deactivates glycogen phosphorylase
    - - Activates glycogen phosphorylase
      - Deactivates glycogen synthase
  - - - Activator
        
        G6P
        
        Glycogen synthase B
        
        cAMP
        
        Phosphorylase kinase B
        
        Calcium
        
        Glycogen synthase A
      - Inhibitor
        
        Glycogen & cAMP
        
        Protein phosphatase
        
        ATP
        
        Phosphorylase kinase B
    - - Activator
        
        Calcium
        
        PKA
        
        cAMP & G6P
        
        Protein phosphatase
      - Inhibitor
        
        Free Hepatic Glucose
        
        Glycogen Phosphorylase A
        
        Glycogen
        
        Phosphorylase kinase B
        
        ATP
        
        PKA
  - - - PIP --> DAG & IP3
        
        IP3 opens Ca+ channel in ER
        
        Ca+ released and binds to calmodulin
        
        Calcium-Calmodulin complex
    - - Activates Ca+ dependent kinase
        
        Inactivates glycogen synthase = no glycogenesis
        
        Activates phosphorylase kinase
        
        Phosphorylation & activation of glycogen phosphorylase = stimulates glycolysis
    - - Stimulates phospholipase C
  - - - Produced when ATP/ADP are depleted
      - Activates glycolysis
- - - - Fasting hypoglycemia
        
        Lactic acidemia
        
        Hyperlipidemia
        
        Most common
  - - - Weakness
        
        Cardiomegaly
        
        Respiratory insufficiency
  - - - Inc. CK, aldolase, myoglobin
  - - - Inc. glycogen
        
        Hepatomegaly
        
        Mild hypoglycemia
  - - - Poor exercise tolerance
        
        Low lactate post exercise
        
        Inc. muscle glycogen
        
        Hemolytic anemia
  - - - Fasting hypoglycemia
        
        Hepatomegaly in infants
        
        Accumulation of polysaccharides
  - - - Hepatomegaly
  - - - Inc. glycogen
        
        Mild hepatomegaly
        
        Hypoglycemia
  - - - Heavily phosphorylated glycogen