Term
|
Definition
| fatty acid + CoA + ATP → fatty acyl CoA |
|
|
Term
People with carnitine/acyltransferase deficiency can't use LCFA for fuel.
1. List two symptoms in adults.
2. How about neonates? |
|
Definition
1. muscle weakness and HYPOKETOSIS
2. arrhythmias, death |
|
|
Term
CAT-I/CPT-I
carnitine acyl transferase I
|
|
Definition
| fatty acyl CoA + carnitine → acylcarnitine + CoA |
|
|
Term
|
Definition
| acylcarnitine + CoA → acyl CoA + carnitine |
|
|
Term
| People with low carnitine, have |
|
Definition
| lipid deposits in muscle, are weak |
|
|
Term
| Primary v. Seconday Carnitine Deficiency |
|
Definition
Primary- congenital deficiency; system or absorption
Secondary-liver disease, diet, hemodialysis, severe infections, pregnancy, burns, trauma |
|
|
Term
|
Definition
(primary)-affects LIVER
hypoglycemia during fast |
|
|
Term
|
Definition
cardiac and skeletal muscle
cardiomyopathy, muscle weakness, prolonged exercise |
|
|
Term
| What inhibits the carnitine shuttle? |
|
Definition
|
|
Term
| What will inhibit Malonyl CoA? |
|
Definition
| Phosphorylation (inhibition) or acetyl CoA carboxylase |
|
|
Term
| What happens to medium/short FACs? |
|
Definition
| activated and oxidized in mitochondira |
|
|
Term
|
Definition
|
|
Term
| What is the importance of succinyl CoA in Oxidation of fatty as? |
|
Definition
| made from oxidation of ODD number chain. Adds 2 Carbons to TCA that can be used for gluconeogen. |
|
|
Term
| Is pyruvate dyhydrogenase reverisble? What does it do? |
|
Definition
no.
pyruvate → acetyl CoA |
|
|
Term
| Review cycles from lecture 2 now. |
|
Definition
|
|
Term
| Regulation of Ketone synthesis |
|
Definition
Promote synthesis....
1. inc. oxidation of f.a's
2. inc. glucagon (inhibit acetyl CoA carboxylase → → activate CPTI)
3. High NADH/ATP will inhibit acetyl co A from entering TCA
|
|
|
Term
| Diabetic Ketoacidosis results in two lab findings... |
|
Definition
ketonemia
ketouria
Explain why. |
|
|
Term
| Excess ketone bodies will promote the release ______ to ______. |
|
Definition
| Insulin to inhibit lipolysis. |
|
|
Term
| What is the physiological role of ketone bodies? |
|
Definition
| They're the preferred fuel of heart and renal-- they are water soluble source of acetyl units. |
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