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9 Cards in this Set
- Front
- Back
FA activation occurs where?
What moves the FA across the membrane> What is the end product released by the FA B-oxidation? |
cytosol - catalyzed by Acyl-CoA synthetase on outer mitochondrial membrane
Carnitine shuttle Acetyl-CoA |
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Pathway of B-oxidation:
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1. oxidized by acyl CoA dehydrogenase
2. hydration by enoyl CoA hydratase 3. oxidation by B-hydroxy Acyl CoA dehydrogenase 4. thiolized by thiolase, Acetyl CoA kicked off |
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Where are very long FA's oxidized?
Effect of glucagon, epi and insulin on FA availibility: Effect of malonyl CoA: |
peroxisomes
glucagon, epi stimulate availability, insulin inhibits inhibits CPT - carnitine shuttle |
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CPT I deficiency:
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hypoketotic, hypoglycemia, hyperammonemia, altered plasma carnitine concentration; affects liver primarily, decreased FA oxidation, ketogenesis
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Most common genetic disorder:
Why is it life threatening during fasting? Zellweger syndrome: |
MCAD - Medium Chain Acyl CoA dehydrogenase deficiency
limits gluconeogenesis - limits Acetyl-CoA formation, causes hypoglycemia absense of peroxisomes - long FA accumulation |
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Explain ketogenesis:
Regulation of ketogenesis: Primary fuel for brain? |
produced in liver when excess Acetyl CoA produced, OA being used for gluconeogenesis
increased FA's, decreased carb metabolism (Atkins diet), gluconeogenesis glucose |
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Pathology of diabetic ketosis:
What happens to Acetyl CoA in muscle, liver? |
untreated diabetes, poor glucose uptake causes overproduction of ketones --> acidosis, ketosis
muscle - TCA cycle --> ATP liver - ketones |
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Three electrolyte causes of coma:
Metabolic situations that cause HG: Causes of increased use: Causes of decreased production: |
imbalance, hypoglycemia, hyperammonemia
increased use of glucose, decreased production insulin overproduction, no FA released decreased synthesis from lactate/AA's, release from glycogen |
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Explain these values in MCAD:
High FA, glucagon hypoglycemia post fasting High ammonia lack of high ketones with high FA, glucagon |
normal response - high FA's, glucagon
high ammonia - AA's used for gluconeogenesis hypoglycemia after prolonged fasting means glycogen breakdown is working lack of high ketones with high glucagon, FA's means problem in catabolism |