Fatty acid Oxidation

Carrier for the transport of fatty acyl CoA into the mitochondrion

Carnitine

Subcellular location of CPT I

Outer mitochondria

Function of CPT I

Transfer acyl to carnitine

Impact of chronic valproate treatment on entry of fatty acyl CoA into mitochondria

Inhibits levocarnitine

Reduce fatty acyl CoA into mitochondria

3 major products of mitochondria B-oxidation

FADH

NADH

Acetyl CoA

Two dehydrogenases and what reaction does each catalyze

Fatty acyl CoA dehydrogenase

- double bond formation

- produce FADH and Beta ketone

Beta hydroxy acyl CoA dehydrogenase

- Converts L-beta-hydroxy acyl-CoA into beta ketoacyl-CoA.

- Oxidizes the hydroxyl group

Produces NADH

How peroxisomes and mitochondria collaborate in the oxidation of very long chain fatty acids

Long chain fatty acids oxidized in peroxisomes

Short chain fatty acids oxidized in the mitochondria

Enzyme responsible for lipolysis

Hormone sensitive lipolipase

Metabolite that inhibits CPT I

Malonyl CoA

Two species of ketone bodies

Acetoacetate and beta hydroxybuturate

Common precursor for each

Acetyl CoA

Organ for ketogenesis

Liver

Subcellular location of ketogenesis

Mitochondria

Physiological significance of ketone body synthesis and utilization

Increased utilization of ketone bodies by tissues reduces need for glucose and spares body proteins from degradation

Diabetic ketoacidosis

Pathogenesis: Insulin deficiency

Breakdown fat instead of blood sugar

High blood ketone level

Reduced blood pH

Life-threatening

Symptoms: Extreme thirst

Frequent urination

Vomiting or stomach pain

Weakness and tiredness

Shortness of breath

Fruity scented breath

Confusion

Whos at risk of diabetic ketoacidosis?

Most common in type I diabetes

Also occurs in people with type 2 diabetes who need insulin

Treatment of diabetic ketoacidosis

Fluids and electrolyte replacement (K)

Insulin therapy

Testing of diabetic ketoacidosis

Blood glucose, ketones, electrolytes, urea nitrogen

Urine analysis: Ketones, urinalysis