In Fabry disease, deficient activity of the enzyme, alpha-galactosidase A, results in the deposits of sphingolipid
degradation Gb3 in lysosomes of various organs including the cornea [2, 5].
Specifically, after a lateral hemisection of the adult rat spinal cord, transient disruption of mature myelin by intraspinal fusion of serum complement proteins and a myelin-specific antibody (anti-galactocerebroside, the major sphingolipid
in myelin) was shown to facilitate axonal regeneration of rubrospinal axons into the caudal lumbar spinal cord .
Igarashi, "Sphingosine-1-phosphate: a platelet-activating sphingolipid
released from agonist-stimulated human platelets," Blood, vol.
Increasing evidence suggests that mitochondria are important intracellular compartments for sphingolipid
metabolism, including sphingomyelin and ceramide .
Tettamanti, " Estimating sphingolipid
metabolism and trafficking in cultured cells using radiolabeled compounds," Methods in Enzymology, vol.
Ceramide is a metabolite in sphingolipid
pathway of the cells that has an ability to promote cell death in tumor cells when produced endogenously in the cells or applied exogenously.
Among the genes involved in sphingolipid
synthesis, SPTLC1 appeared to be overexpressed (>3-fold) whereas the expression of SGPL1 appeared to be downregulated at about 1/20th of cutaneous expression.
Symolon et al., "Ceramides and other bioactive sphingolipid
backbones in health and disease: lipidomic analysis, metabolism and roles in membrane structure, dynamics, signaling and autophagy," Biochimica et Biophysica Acta-Biomembranes, vol.
Booth, "Vitamin K and sphingolipid
metabolism: Evidence to date," Nutrition Reviews, vol.
Ceramides (Cer) belongs to the sphingolipid
family and are formed by a sphingosine and a fatty acid group linked by an amide bond.
As for higher eukaryotes, also in yeast, our information about the effect of sphingolipid
metabolism on mitochondrial function and death is limited to ceramide levels [27, 28].
Thirteen biomarkers involved in NO production, inflammatory state, and vascular smooth muscle cells (VSMCs) apoptosis and proliferation, the main metabonomic pathways, were sphingolipid
metabolism (sphinganine, lysosphingomyelin, and ceramide), glycerophospholipid metabolism (phosphatidylcholines, phosphatidylethanolamine, and lysophosphatidylcholines), arginine and proline metabolism (l-proline, citrulline), tryptophan metabolism (xanthuiulrenic acid, l-kynurenine, and l-tryptophan), arachidonic acid metabolism (leukotriene D4), and linoleic acid metabolism (gammalinolenic acid), which could well explain the mechanism of physiological and pathological state of hypertension and the potential therapeutic effects of those prescriptions [12-14].