Test 2 Lecture 25

Question 1

Metabolic shifts and major changes in the use of carbohydrates, amino acids, and fatty
acids occur during prolonged ___

Answer 1

starvation and diseases.

Question 2

Deregulation in metabolism of stored ___ and fatty acids are cause of important
metabolic/genetic diseases.

Answer 2

glycogen

Question 3

____ represents a class of diseases associated with defective fatty acid transport or catabolism.

Answer 3

Hypoketotic hypoglycemia

Question 4

High insulin induces ___, activates Pyruvate dehydrogenase and thus conversion of Pyruvate to Acetyl CoA. Insulin also inhibits ____ and activates fatty acid synthesis.

Answer 4

glycolysis,
lipolysis

Question 5

When there is high blood glucose and high insulin, the mitrocondria will shift to produce ___

Answer 5

Fatty acids

Question 6

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.

Answer 6

glycolysis
lipolysis

Question 7

When there is low blood glucose and high glucagon, mitochondria will shift to form ___ over ___

Answer 7

glucose
fatty acid synthesis

Question 8

Metabolic shift to ___ utilization and diversion of carbon skeletons for gluconeogenesis during starvation

Answer 8

fatty acid

Question 9

ketone usage during starvation

Answer 9

will start using glucose, when that runs out will use ketone bodies

Question 10

____ : spectrum of diseases with defects in different steps of fatty acid transport and utilization.

Answer 10

Hypoketotic Hypoglycemia:

Question 11

Acyl CoA dehydrogenase deficiency (often fatal), which limits the oxidation of fatty acids
in mitochondria by ____.

Answer 11

beta oxidation

Question 12

Carnitine deficiency or acyl carnitine transferase deficiency, both of which limit the ____ transport into mitochondria.

Answer 12

fatty acid

Question 13

The blood glucose level of the patient drops dramatically, while serum___ levels
go up in 2 hrs after a meal.

Answer 13

fatty acid

Hypoketotic Hypoglycemia:

Question 14

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.

Answer 14

Reye-like

Question 15

graph of Hypoketotic Hypoglycemia

glucose will ___ and fatty acid will ___

Answer 15

rapidly drop
rapidly increase

Question 16

Defects in fatty acid catabolism pose a major problem for ____ blood glucose at the same time maintaining cellular energy levels.

Answer 16

buffering

Question 17

Hypoketotic Hypoglycemia can be treated by ___

Answer 17

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.

Question 18

Hypoketotic Hypoglycemia is in sharp contrast to hypoglycemia in lactating cows which
causes ____

Answer 18

ketoacidosis.

Question 19

Muscle glycogen phosphorylase deficiency. Unable to mobilize glucose for muscle activity; Rigorous muscle activity will result in phospho-creatine breakdown and alkalosis.

Answer 19

McArdle’s Disease

Question 20

deficiency in lysosomal acid alpha-1 glycosidase; deposition of glycogen in the liver and muscle lysosomes.

Answer 20

Pompe’s disease

Question 21

Liver phosphorylase deficiency. Vastly enlarged liver and liver malfunction, inability to buffer blood glucose in response to glucagon
release. Hypoglycemia, mental retardation, etc

Answer 21

Her’s disease

Question 22

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.

Answer 22

Lactic acidosis

Question 23

Lactic Acidosis: Increased glycolysis coupled with reduced oxidaitve metabolism of pyruvate causes accumulation of lactate which leads to ___ blood pH.

Answer 23

lower

Question 24

Increased ketone body production, diabetic acidosis, chronic starvation, lactation, chronic alcohol consumption.

Answer 24

keto acidosis

Question 25

to counter acidosis the kidneys excrete

Answer 25

NH⁺₄ in exchange for HCO^-₃