Enzymes of sphingolipid metabolism

Hanada K. (2003). Serine palmitoyltransferase, a key enzyme of sphingolipid metabolism. Biochim. Biophys. Acta 1632 16-30. 

The molecular identification of several of the key enzymes of sphingolipid metabolism and some of their molecular targets has also allowed for the... 

Hannun, YA, Luberto, C and Argraves, KM (2001) Enzymes of sphingolipid metabolism from modular to integrative signaling. Biochemistry, 40,4893-4903. 

Delgado A, Casas J, Llebaria A, Abad JL, Fabrias G. Inhibitors of sphingolipid metabolism enzymes. Biochim. Biophys. Acta 2006 1758 1957-1977. 

Figure 8 Outline of the sphingolipid biosynthetic pathway. Natural and synthetic inhibitors of sphingolipid-metabolizing enzymes are shown adjacent to the inverted T bars.

Delgado, A., Casas, J., Llebaria, A., Abad, J.L., and Fabrias, G., Inhibitors of sphingolipid metabolism enzymes, Biochim Biophys Acta, 1758 (2006) 1957-1977. 

Gaucher s disease is an inborn error of sphingolipid metabolism caused by a deficiency of the lysosomal enzyme, acid p-glucosidase (EC 3.2.1.45) which is responsible for cleaving glucosylceramide (GlcCer) into glucose and... 

Vitamin B6 occurs naturally in three related forms pyridoxine (6.26 the alcohol form), pyridoxal (6.27 aldehyde) and pyridoxamine (6.28 amine). All are structurally related to pyridine. The active co-enzyme form of this vitamin is pyridoxal phosphate (PLP 6.29), which is a co-factor for transaminases which catalyse the transfer of amino groups (6.29). PLP is also important for amino acid decarboxylases and functions in the metabolism of glycogen and the synthesis of sphingolipids in the nervous system. In addition, PLP is involved in the formation of niacin from tryptophan (section 6.3.3) and in the initial synthesis of haem. 

The chemical structure of fumonisin (Fig. 5.IB) is remarkably similar to that of the sphingoid bases sphinganine (Sa) and sphingosine (So), and fumonisin has been shown to inhibit the enzyme ceramide synthase in de novo sphingolipid metabolism (Wang et al., 1991). Ceramide synthase... 

Inhibition of other enzymes in the sphingolipid metabolic pathway by natural products... 

Chatteijee, D., Mukheijee, S., Smith, M.G., and Das, S.K. (2004). Role of sphinomyelinase in the environmental toxin induced apoptosis of pulmonary cells. In In Lipids Sphingolipid Metabolizing Enzymes, eds. Dipak, Haidar, and Salil K Das, Research Signpost, pp. 117-139, Trivandum, Kerala, India. 

Each lysosomal storage disease (see p. 49) is caused by a hereditary deficiency of an enzyme required for the degradation of a specific metabolite. Several lysosomal storage diseases are associated with sphingolipid metabolism. Most of... 

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