Unlike carbohydrate fuels, which enter the body primarily as glucose or sugars that are readily converted to glucose , lipid fuels are heterogeneous with respect to chain length, branching, and unsaturation. The catabolism of fats is primarily a mitochondrial process, but also occurs in peroxisomes. Using a variety of chain-length-specific transport processes and catabolic enzymes, the primary pathways of catabolism of fatty acids involve their oxidative degradation in two-carbon units - a process known as β-oxidation, which produces acetyl-CoA. In muscle, the acetyl-CoA units are used for ATP production in the mitochondria, whereas in liver the acetyl-CoA is catabolized to ketone bodies, primarily acetoacetate and β-hydroxybutyrate, that are exported for energy metabolism in peripheral tissue.
|
- Compare the metabolism of acetyl-CoA in liver and muscle. Explain why the liver produces ketone bodies during gluconeogenesis. What prevents hepatic oxidation of acetyl-CoA?
- Review the merits of carnitine usage as a performance enhancer during exercise and as a supplement for geriatric patients.
- Review the current use and mechanism of action of peroxisome proliferator drugs for treatment of dyslipidemia and diabetes.
|
page 197 |  | page 198 |
Atar D, Spiess M, Mandinova A, Cierpka H, Noll G, Luscher TF. Carnitine - from cellular mechanisms to potential clinical applications in heart disease. Eur J Clin Invest 1997;27:973-976.
Full article
|
Depreter M, Espeel M, Roels F. Human peroxisomal disorders. Microsc Res Tech 2003;61:203-223.
Full article
|
Eaton S, Bartlett K, Pourfarzam M. Mammalian mitochondrial β-oxidation. Biochem J 1996;320:345-357.
|
Freeland BS. Diabetic ketoacidosis. Diabetes Educator 2003;29:384-395.
|
McGarry JD, Brown NF. The mitochondrial carnitine palmitoyltransferase system. From concept to molecular analysis. Eur J Biochem 1997;244:1-14.
Full article
|
Mitchell GA, Kassovska-Bratinova S, Boukaftane Y, et al. Medical aspects of ketone body metabolism. Clin Invest Med 1995;18:193-216.
|
Rinaldo P, Raymond K, al-Odaib A, Bennett MJ. Clinical and biochemical features of fatty acid oxidation disorders. Curr Opin Pediatr 1998;10:615-621.
|
Singh I. Biochemistry of peroxisomes in health and disease. Mol Cell Biochem 1997;167:1-29.
Full article
|
Swink TD, Vining EP, Freeman JM. The ketogenic diet: 1997. Adv Pediatr 1997;44:297-329.
|
Wanders RJ, Jansen GA, Lloyd MD. Phytanic acid alpha-oxidation, new insights into an old problem: a review. Biochim Biophys Acta 2003;1631:119-135.
|
Wierzbicki AS, Lloyd MD, Schofield CJ, Feher MD, Gibberd FB. Refsum's disease: a peroxisomal disorder affecting phytanic acid alpha-oxidation. J Neurochem 2002;80:727-35.
Full article
|
Wood PA. Defects in mitochondrial beta-oxidation of fatty acids. Curr Opin Lipidol 1999;10:107-112.
Full article
|
|