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47 Cards in this Set

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