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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
Pathway of B-oxidation:
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
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
CPT I deficiency:
hypoketotic, hypoglycemia, hyperammonemia, altered plasma carnitine concentration; affects liver primarily, decreased FA oxidation, ketogenesis
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
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
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
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
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