Fatty Acid Disorders pg 112-114

Question 1

What does carnitine normally do? Systemic 1° carnitine deficiency what kind of problem? What kind of problems does it manifest?

Answer 1

transports Acyl and fatty acid group into the mitochondria

inability to transport fatty acid-acyl group

Weakness, Hypoketotic Hypoglycemia

Question 2

MCAD what kind of problem? What kind of problems does it manifest? What is the major complication and treatment?

Answer 2

Inability to breakdown fatty acid

Weakness, Hypoketotic Hypoglycemia

Complication: sudden death

Tx: Avoid fasting

Question 3

In the Liver, fatty acid and amino acids are metabolized to _______ and _________?

Answer 3

acetoacetate and β-hydroxybutyrate (to be used in muscle and brain).

Question 4

In prolonged starvation and DKA, what is depleted for gluconeogenesis?

Answer 4

oxaloacetate

Question 5

In alcoholism, excess NADH shunts _____ to _______

Answer 5

oxaloacetate to malate

Question 6

Alcoholism, prolonged starvation and DKA all lead to build up of _______ and shunt so that _______ is produced?

Answer 6

acetyl-coA

Ketone bodies

Question 7

How do you know the patient has high quantities of ketones being produced? Will a urine test be + for β-hydroxybutyrate?

Answer 7

Breathe=fruit smell

No

Question 8

What are the sequence of used fuel in the body during exercise?

Answer 8

Stored ATP=> creatinine phosphate=>anaerobic=>aerobic

Question 9

During fasting, what are the priorities (name 2)?

Answer 9

To the brain and RBC

Conserve proteins

Question 10

During fasting, what are the two hormones converting glycogen to glucose?

Answer 10

Glucagon and Epinephrine.

Question 11

During fasting, what is major source of fuel?

Answer 11

Hepatic glycogenolysis (major)

Question 12

During the first 3 days of fasting, blood glucose is maintained by?

Answer 12

Hepatic glycogenolysis

Adipose release of FFA from Muscle and liver

Hepatic gluconeogenesis from peripheral
tissue lactate and alanine, glycerol and propionyl- CoA

Question 13

Is glycogen storage depleted after the 1st day?

Answer 13

Yes

Question 14

RBC’s lack ________ and therefore they cannot use ketones

Answer 14

mitochondria (because without mitochondria=> cannot enter Krebs cycle)

Question 15

Starvation after day 3, what is main source of energy for body and brain? Does the amount of excess storage determine survival of the patient?

Answer 15

Body: adipose
Brain: Ketones

Yes

Question 16

Rate limiting enzyme of cholesterol synthesis? What stimulates it? Inhibits?

Answer 16

HMG-CoA reductase
Stimulation: insulin
Inhibition: statin