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21 Cards in this Set

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Glycogen Phosphorylase
-Glycogen + Pi---> glucose 1-P + glycogen (minus one sugar)
-Sterically inhibited 4 sugars away from branching point
-Phosphorolysis
-alpha 1,4-linkages
Debranching enzyme-2 activities
-Transferase activity, takes off most of the branch and moves it to the end of the molecule.
1. Transferase: removes branches
2. Alpha-1,6 glucosidase: removes last glucose molecule (breaks alpha-1,6) with water
Glucose 1-P--->glucose-6-P
Phosphoglucomutase
Glucose-6-P--->glucose--->blood
Glucose-6-Phosphatase
Glycogen ---> Glucose-1-P
Glycogen phosphorylase
Regulation of Glycogen Breakdown
AMP +
ATP -
Glucose 6-P -
Ca +2 +
Hormonal control
Epinephrine + glucagon--->adenylate cyclase (ATP)--->Cyclic AMP--->protein kinase--->Phosphorylase Kinase
Inactive, unphosphorylated, regulated Glycogen Phosphorylase --->Inactive, phosphorylated, unregulated Glycogen Phosphorylase
Phosphorylase Kinase
Glycogen Phsophorylase (inactive, phosphorylated, unregulated)--->Glycogen Phosphorylase (active, phosphorylated, unregulated)
Glucose
Glycogen Synthase (active, unphosphorylated) ---> Glycogen Synthase (inactive, phosphorylated)
Protein Kinase
Glycogen Synthase (inactive phosphorylated) ---> Glycogen Synthase (active, unphosphorylated )
Phosphatase
Glycogen Phosphorylase (inactive, unphosphorylated) regulation
AMP +
ATP -
GTP -
Glycogen Phosphorylase (active, phosphorylated)regulation
Insensitve to cellular effectors, except glucose -
Glycogen Synthase (inactive, phosphorylated)
G-6-P +
Von Gierke's Disease
Deficient in glucose 6-Phosphatase (can't break down glycogen)
-Increased amounts of glycogen in liver
-Liver enlargment, hypoglycemia, ketosis, hyperuricemia, hyperlipemia
-correct by giving simple sugars
Pompe's Disease
Deficient in alpha 1,4-glucosidase (which removes end glucose molecules from glucose stores)
-Increases amount of glycogen
-Cardiorespiratory failure, death by age 2
Cori's Disease
-Deficient in amylo-1,6-glucosidase (debranching enzyme)
-Increase amt of glycogen, but shorter outer branches
-Still have some access to glycogen stores
-Milder form of Von Gierke's Disease
Andersen's Disease
-Deficient in branching enzyme (alpha 1,2 to alpha 1,6)
-Normal amt of glycogen, very long single chains (no branching)
-Inflammatory response bc thinks long chains are a foreign substance
-Cirrhosis of liver, death by age 2
McArdle's Disease
-Defective Phosphorylase
-Glycogen normal, but increased amts of it
-Limited ability to perform strenuous exercise bc of painful muscle cramps
-In muscle
Hers' Disease
-Defective Phosphorylase
-Increase amount of glycogen
-in liver
Type VIII
-Defective phosphorylase kinase (turns tense structure into tense phosphorylate form)
-Increased amt of glycogen
-Mild liver enlargment, mild hypoglycemia
-Sex-linked trait