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10 Cards in this Set

  • Front
  • Back
Most common glycogen storage diseases:
-Von Gierke's
-Pompe's
-Cori's
-McArdle's
Cause of Pompe's disease
Deficiency of a-1,4-glucosidase
What is a-1,4-Glucosidase used for?
A glycogen degradation pathway in lysosomes - part of normal housekeeping, not metabolism.
What glycogen storage disease is most common?
Von Gierke
Cause of Von Gierke:
-organs affected
-glycogen nature
Deficient in Glu-6-phosphatase (not present in muscle anyway)
-Liver/kidney
-Glycogen normal, backed up so increased in amount.
Cause of Pompe:
-organs affected
-glycogen nature
Deficient a-1,4-glucosidase
-housekeeping lysosome enzyme - affects all organs
-normal glycogen, incr. amt
Cause of Cori disease:
-organs affected
-glycogen nature
Deficient Amylo-1,6-glucosidase DEBRANCHING ENZYME (bifunctnl)
-muscle and liver affected
-Short outer branches on glycogen
Cause of McArdle disease:
-organs affected
-glycogen nature
Lack Glycogen Phosphorylase
-Muscle only
-Normal glycogen, increased amt
Andersen disease
-organs affected
-glycogen nature
Deficient branching enzyme
-liver/spleen only
-very long outer branches
Hers disease
-organs affected
-glycogen nature
Deficient Glycogen Phosphorylase
-liver only
-increased amount