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47 Cards in this Set
- Front
- Back
White muscle
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Anaerobic
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Red slow twitch
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Aerobic
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Glycoge"s core protein
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Glycogenin
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Rate limiting step of glycogen synthesis
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Glycogen synthase
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Glycogen synthase in liver: activation inhibition?
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Insulin, Inhibited by glucagon, epinephrine
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Glycogen synthase in Skeletal Muscle: activation inhibition?
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Insulin, inhibited by epinephrine
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Glycogen phosphorylase in in liver: activation inhibition?
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Activation: Glucagon, epi Inhibition: Insulin
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Glycogen phosphorylase in Skeletal muscle: activation inhibition?
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Inhibited:insulin ATP, Activated: Epinephrine, AMP, Ca2+
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Von Gierke
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Glucose 6-Phosphatase deficiency
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Pompe
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Lysosomal a1-4 glucosidase
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Cori
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Debranching
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Anderson
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Branching enzyme
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Mcardle
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Muscle phosphorylase
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Hers
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Hepatic phosphorylase
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Cardiomegaly
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Pompe
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Death at 2 years
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Anderson, Pompe
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Myoglobinuria
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McArdle's
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Severe hypoglycemia
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Von Gierke's
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Mild hypoglycemia
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Cori, Hers
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Infantile hypotonia
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Cori's
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Muscle weakness
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McArdles's
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Protuding abdomen, emaciated, doll ike facies
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Von-Gierke
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Glycogen like material in inclusion bodies
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Pompe
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Glycogen structure abnormal
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Cori, Anderson
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Why mild hypoglycemia in Hers?
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Gluconeogenisis still works
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Does administration of fructose, galactose epinehrine glucagonlead to increase in blood glucose of Von Gierke Disease?
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No
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Improves at second wind, 10-15 minutes after exercise?
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McArdle
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McArdles improves by sucrose drink?
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Yes, increses glucose available
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Similar to tay sachs/I cell
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pompe
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Gluconeogeneisis sole source of glucose
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after 24 hours
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ketogenic aa?
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Leucine, lysine
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Can glucose be produced by acetyl CoA/fatty acids?
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No
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Can glucose be produced by Odd chain fattty aciD?
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Yes, they yeild small amount of propionyl-CoA
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If acytl CoA cant produce glucose, its fate?
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Fatty acid synthesis
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Pyruvate Carboxylase and pyruvate dehydrogenase controlled by..?
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Acetyl CoA
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Increased NADH, consequences
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Increased lactate, malate, Glycerol 3-P
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The amount of NADH in a cell is very tiny.. example
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MArathon runner, drinks beer after exercise, dies of lactate acidosis
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Functions of HMP shunt
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1. Production NADPH 2) Ribose 5-Phosphate
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G6PDH induced by
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Insulin, NADp
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G6PDH inhibited by
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NADPH
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The only thiamine requiring enzyme
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Transketolase
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Functions of NADPH
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Biosynthesis, ROS, PMN bactericidal activity
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Biosynthesis of..
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Chlestrol fatty acid, nucleosides
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ROS..
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Maintaining supply of reduced Glutathione
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BActericidal acticity..
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In PMN, NADPH oxidase uses it to generate H2O2
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G6PDH deficiency
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Hemolytic anemia, Heinz Bodies, Immunodeficiency
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CGD
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NADPH oxidase deficiency
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