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7 metabolism of complex lipid.ppt
- 2. Biosynthesis of phospholipids Degradation of phospholipids Disorders of complex lipid metabolism
- 5. 1960
- 13. Deficiency of Glucocerbrosidase or ฮฒ-glucosidase Gaucherโs disease Deficiency of Spingomyelinase Niemann-Pick disease
- 14. Deficiency of ฮฒ-galactosidase Generalized gangliosidosis GM1 ganglioside
- 15. Deficiency of hexosaminidase A Tay-Sachs disease
- 16. Deficiency of hexosaminedase B Sandhoffโs disease
- 17. Deficiency of ฮฑ-galactosidase Fabryโs disease
Editor's Notes
- The central importance of cytidine nucleotides in lipid biosynthesis was discovered by Eugene P. Kennedy in the early 1960s.
- Synthesis of phosphatidylcholine, phosphatidylethanolamine, and phosphatidylserine. The multiple pathways reflect the importance of phospholipids in membrane structure. For example, phosphatidylcholine (PC) can be synthesized from dietary choline when it is available. If choline is not available, PC can be made from dietary carbohydrate, although the amount synthesized is inadequate to prevent choline deficiency. SAM is S-adenosylmethionine, a methyl group donor for many biochemical reactions (see Chapter 40).
- B. Degradation of Glycerophospholipids Phospholipases located in cell membranes or in lysosomes degrade glycerophospholipids. Phospholipase A1 removes the fatty acyl group on carbon 1 of the glycerol moiety, and phospholipase A2 removes the fatty acid on carbon 2 (Fig. 33.31). The C2 fatty acid in cell membrane phospholipids is usually an unsaturated fatty acid, which is frequently arachidonic acid. It is removed in response to signals for the synthesis of eicosanoids. The bond joining carbon 3 of the glycerol moiety to phosphate is cleaved by phospholipase C. Hormonal stimuli activate phospholipase C, which hydrolyzes PIP2 to produce the second messengers DAG and inositol triphosphate (IP3). The bond between the phosphate and the head group is cleaved by phospholipase D, producing phosphatidic acid and the free alcohol of the head group.
- Globosides are glycolipids containing multiple sugar units Kidney and RBC Determinant of blood group