Test 2 Lecture 25 Flashcards
Metabolic shifts and major changes in the use of carbohydrates, amino acids, and fatty
acids occur during prolonged ___
starvation and diseases.
Deregulation in metabolism of stored ___ and fatty acids are cause of important
metabolic/genetic diseases.
glycogen
____ represents a class of diseases associated with defective fatty acid transport or catabolism.
Hypoketotic hypoglycemia
High insulin induces ___, activates Pyruvate dehydrogenase and thus conversion of Pyruvate to Acetyl CoA. Insulin also inhibits ____ and activates fatty acid synthesis.
glycolysis,
lipolysis
When there is high blood glucose and high insulin, the mitrocondria will shift to produce ___
Fatty acids
High glucagon inhibits ___, stimulates gluconeogenesis, and also induces ____thus increasing serum fatty acid levels. Under this condition, available Pyruvate is used for oxaloacetate (Oaa) synthesis and gluconeogenesis.
glycolysis
lipolysis
When there is low blood glucose and high glucagon, mitochondria will shift to form ___ over ___
glucose
fatty acid synthesis
Metabolic shift to ___ utilization and diversion of carbon skeletons for gluconeogenesis during starvation
fatty acid
ketone usage during starvation
will start using glucose, when that runs out will use ketone bodies
____ : spectrum of diseases with defects in different steps of fatty acid transport and utilization.
Hypoketotic Hypoglycemia:
Acyl CoA dehydrogenase deficiency (often fatal), which limits the oxidation of fatty acids
in mitochondria by ____.
beta oxidation
Carnitine deficiency or acyl carnitine transferase deficiency, both of which limit the ____ transport into mitochondria.
fatty acid
The blood glucose level of the patient drops dramatically, while serum___ levels
go up in 2 hrs after a meal.
fatty acid
Hypoketotic Hypoglycemia:
If not properly treated these diseases are fatal. Some forms of sudden infantile death
and ___ syndrome are thought to be caused by deficiency in fatty acid
metabolism.
Reye-like
graph of Hypoketotic Hypoglycemia
glucose will ___ and fatty acid will ___
rapidly drop
rapidly increase
Defects in fatty acid catabolism pose a major problem for ____ blood glucose at the same time maintaining cellular energy levels.
buffering
Hypoketotic Hypoglycemia can be treated by ___
feeding short chain fatty acids such as butyric acid and palmitic acid.
Note that this condition is in sharp contrast to hypoglycemia in lactating cows which
causes ketoacidosis.
Hypoketotic Hypoglycemia is in sharp contrast to hypoglycemia in lactating cows which
causes ____
ketoacidosis.
Muscle glycogen phosphorylase deficiency. Unable to mobilize glucose for muscle activity; Rigorous muscle activity will result in phospho-creatine breakdown and alkalosis.
McArdle’s Disease
deficiency in lysosomal acid alpha-1 glycosidase; deposition of glycogen in the liver and muscle lysosomes.
Pompe’s disease
Liver phosphorylase deficiency. Vastly enlarged liver and liver malfunction, inability to buffer blood glucose in response to glucagon
release. Hypoglycemia, mental retardation, etc
Her’s disease
Increased glycolysis coupled with reduced oxidaitve metabolism of pyruvate causes accumulation of lactate which leads to lower blood pH: Pyruvate dehydrogenase deficiency, deficiency of liver specific Lactic Acid Dehydrogenase, hepatic cancer, increased muscle glycolysis, impaired Cori cycle.
Lactic acidosis
Lactic Acidosis: Increased glycolysis coupled with reduced oxidaitve metabolism of pyruvate causes accumulation of lactate which leads to ___ blood pH.
lower
Increased ketone body production, diabetic acidosis, chronic starvation, lactation, chronic alcohol consumption.
keto acidosis
to counter acidosis the kidneys excrete
NH+4 in exchange for HCO-3
