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10 Cards in this Set
- Front
- Back
- 3rd side (hint)
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Presentation:
-Muscle aches and fatigue following exercise -Elevated free fatty acids in blood -Reduced Ketone production -Hypoglycemia |
Defect:
Carnitine or Carnitine acyltransferase deficiency |
Can't transport long-chain fatty acids into mitochondria
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Presentation:
-Hypoglycemia -vomiting -lethargy -minimal ketone production |
Defect:
MCAD deficiency |
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Presentation
-Adrenocortical insufficiency -Mental retardation -Spasticity |
Defect:
Defective peroxisomal oxidation of very long chain fatty acids ("Adrenoleukodystrophy"; X-linked) |
Very long chain fatty acids build up in tissue
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Presentation:
-Retinitis pigmentosa -Dry scaly skin -Ataxia -Elevated protein in CSF |
Defect:
Inability to degrade phytanic acid (branched chain FA found in dairy) |
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Rate limiting step of FA oxidation
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Carnitine Acyltransferase I
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Inhibited by Malonyl-CoA
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Rate limiting step of FA release
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Hormone sensitive lipase
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Insulin inhibits
Glucagon activates |
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Rate limiting step of FA synthesis
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Acetyl-CoA carboxylase
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Insulin and Citrate activate
Glucagon, Epinephrine, High AMP, Palmitate inhibit |
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How is Acetyl-CoA brought to the cytosol to start FA synthesis
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Citrate shuttle
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Produces NADPH
Insulin activates Citrate Lyase (Citrate --> Acetyl CoA + OAA in cytosol) |
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What are the two sources of propionyl-CoA
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Fatty Acid Oxidation
Methionine, Valine, Isoleucine |
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What are the two fates of HMG-CoA
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(1) Ketone bodies (made in mt)
(2) Cholesterol via HMG-CoA Reductase (insulin activates, glucagon inhibits) |
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