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15 Cards in this Set
- Front
- Back
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4 glycogen storage diseases
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Von Gierke's Disease (Type 1)
Pompe's Disease (Type II) Cori's Disease (Type III) McArdle's Disease (Type V) |
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Deficient glucose-6-phosphatase
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Von Gierke's
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Deficient glycogen phosphorylase
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McArdle's
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Deficient alpha-1,6-glucosidase
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Cori's (is a debranching enzyme)
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Deficient lysosomal alpha-1,4-glucosidase
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Pompe's disease
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Deficient acid maltase
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Pompe's disease (another name for the same enzyme)
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In which is gluconeogenesis intact?
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Cori's
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Painful muscle cramps, myoglobinuria with strenuous exercise
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McArdle's
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3 systems affected by Pompe's?
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Heart, liver, muscle
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What distinguishes Type I from Type III?
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Type III is milder, blood lactate levels are normal
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Hypoglycemia and hyperketossi
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McArdle's
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What does glycogen phosphorylase do?
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Cleaves alpha-1,4-linkage
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Lactic acidosis
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Von Gierke's
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All four result in
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Accumulation of glycogen within cells
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Severe fasting hypoglycemia, increased glycogen in liver, hepatomegaly, gout
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Von Gierke's
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