KinasesKinaseCeramidesCDaseASAH1SphingolipidAcid ceramidase activityPhosphorylationAlkalineAliphatic aminoCeramide-1-phosphatePhosphateLipidBackboneFatty acidsNeutralEnzymeFarberSMaseBasesFormFamilyCellNormalCeramide to sphingosineSphingolipidsAcidAlkalineSphingolipid metabolism1.23MetabolismSphingoid basesHydrolysisPhytosphingosineFacilitatesDegenerationActivity
Kinases5
- Sphingosine can be phosphorylated in vivo via two kinases, sphingosine kinase type 1 and sphingosine kinase type 2. (foodb.ca)
- Sphingosine produces S1P through phosphorylation by sphingosine kinases 1 (SphK1) and sphingosine kinases 2 (SphK2) . (cusabio.com)
- S1P is generated intracellularly by two sphingosine kinases (SphK1, SphK2) and is exported out of cells by Spinster 2 (Spns2) to exert its effects through activation of five specific cell surface S1PRs in autocrine or paracrine manners. (sphingolipidclub.com)
- Ceramide could be metabolized to various other bioactive sphingolipid types, phosphorylated by ceramide kinase (CERK) to ceramide-1-phosphate (C1P), or hydrolysed to sphingosine, which is normally after that phosphorylated to sphingosine-1-phosphate (S1P) by sphingosine kinases (SphKs). (moonphase2018.com)
- ASAH1 and sphingosine kinases (SPHK I/II) blockade significantly reduced IL-6, but not IFNβ, after STING activation. (unisa.it)
Kinase6
- Sphingosine may be phosphorylated by sphingosine kinase 1/2 (SPHK1/SPHK2) to form sphingosine-1-phosphate (S1P), which has a prosurvival role and is critical for immunomodulation ( 1 , 4 , 5 ) ( Figure 2 ). (frontiersin.org)
- Further, activities of acid sphingomyelinase (ASM), acid ceramidase, and sphingosine kinase were measured by ultraperformance liquid chromatography. (uni-potsdam.de)
- A significant increase of ASM and sphingosine kinase activities, as well as lipid formation, was observed in GO-derived OFs. (uni-potsdam.de)
- S1P produced from the transformation of ceramide to sphingosine by ceramidase, and it is a product from the phosphorylation of sphingosine by sphingosine kinase isoenzymes, has an important function in peripheral and central sensitization. (gasyblog.com)
- Sphingosine kinase 1 promotes tumour cell migration and invasion via the S1P/EDG1 axis in hepatocellular carcinoma [J].2012,32(2). (wanfangdata.com.cn)
- Identification of sphingosine kinase 1 (SphK1) as a primary target of icaritin in hepatocellular carcinoma cells. (wanfangdata.com.cn)
Ceramides11
- Acid ceramidase breaks down fats called ceramides into a fat called sphingosine and a fatty acid. (medlineplus.gov)
- Ceramidase (CDase) hydrolyzes the amide bond in ceramides to yield free fatty acid and sphingosine. (tno.nl)
- Lysosomal ceramidase that hydrolyzes sphingolipid ceramides into sphingosine and free fatty acids at acidic pH (PubMed:10610716, PubMed:7744740, PubMed:15655246, PubMed:11451951). (icr.ac.uk)
- Ceramides, sphingosine, and its phosphorylated form sphingosine-1-phosphate are bioactive lipids that mediate cellular signaling pathways regulating several biological processes including cell proliferation, apoptosis and differentiation (PubMed:10610716). (icr.ac.uk)
- Also catalyzes the reverse reaction allowing the synthesis of ceramides from fatty acids and sphingosine (PubMed:12764132, PubMed:12815059). (icr.ac.uk)
- By regulating the levels of ceramides, sphingosine and sphingosine-1-phosphate in the epidermis, mediates the calcium-induced differentiation of epidermal keratinocytes (PubMed:17713573). (icr.ac.uk)
- By regulating the intracellular balance between ceramides and sphingosine, in adrenocortical cells, probably also acts as a regulator of steroidogenesis (PubMed:22261821). (icr.ac.uk)
- Within lysosomes, acid ceramidase breaks down fats called ceramides. (encyclopedia.pub)
- When ceramides need to be replaced, they travel to lysosomes where acid ceramidase breaks them down into a fat called sphingosine and a fatty acid. (encyclopedia.pub)
- The decrease in acid ceramidase activity leads to inefficient breakdown of ceramides and impaired production of its breakdown products sphingosine and fatty acids. (encyclopedia.pub)
- The increase in ceramides and reduction in sphingosine and fatty acids likely play a role in the development of the features of SMA-PME, but the exact mechanism is unknown. (encyclopedia.pub)
CDase1
- Ceramidase (CDase) hydrolyses the N-acyl linkage of the sphingolipid ceramide. (tno.nl)
ASAH15
- The ASAH1 gene provides instructions for making an enzyme called acid ceramidase. (medlineplus.gov)
- Variants in the ASAH1 gene lead to severe reduction in acid ceramidase, typically to below 10 percent of normal. (medlineplus.gov)
- Alves MQ, Le Trionnaire E, Ribeiro I, Carpentier S, Harzer K, Levade T, Ribeiro MG. Molecular basis of acid ceramidase deficiency in a neonatal form of Farber disease: identification of the first large deletion in ASAH1 gene. (medlineplus.gov)
- The ASAH1 gene mutations that cause SMA-PME result in a reduction of acid ceramidase activity to a level less than one-third of normal. (encyclopedia.pub)
- The mRNA levels of ceramidase (ASAH1), S1P precursor enzyme, and STING were inversely correlated in healthy lung tissues, but positively correlated in tumor tissues. (unisa.it)
Sphingolipid3
- Acid ceramidase (Ac) is part of the sphingolipid metabolism and responsible for the degradation of ceramide. (elifesciences.org)
- In mammals, the sphingolipid signaling pathway refers to the process by which ceramide (Cer) and sphingosine-1-phosphate (S1P), the hydrolytic products of sphingolipid, functions in a variety of cellular signaling pathways. (cusabio.com)
- Sphingosine 1-phosphate (S1P) is a pleiotropic bioactive sphingolipid metabolite that regulates numerous processes important for inflammation and cancer. (sphingolipidclub.com)
Acid ceramidase activity2
- It is unclear whether the level of acid ceramidase activity is related to the severity of the disorder. (medlineplus.gov)
- The reduction in acid ceramidase activity associated with SMA-PME is less than what occurs in another condition called Farber lipogranulomatosis (described above). (encyclopedia.pub)
Phosphorylation1
- In addition, I will discuss recent studies demonstrating that SphK1 is recruited to sphingosine-enriched endocytic vesicles and that phosphorylation of sphingosine to S1P by SphK1 is involved in endocytic membrane trafficking and autophagy and in the crosstalk between endocytosis and autophagy. (sphingolipidclub.com)
Alkaline1
- Alkaline ceramidase 3 promotes growth of hepatocellular carcinoma cells via regulating S1P/S1PR2/PI3K/AKT signaling [J].2018,214(9). (wanfangdata.com.cn)
Aliphatic amino2
- Sphingolipids are a class of lipids containing a backbone of sphingoid bases, which are a set of aliphatic amino alcohols that includes sphingosine. (wikipedia.org)
- Sphingolipids are key structural components of cellular membranes containing a backbone of sphingosine (aliphatic amino alcohol) as the base of their structures. (frontiersin.org)
Ceramide-1-phosphate1
- The addition of a phosphate group to sphingosine or ceramide yields sphingosine-1-phosphate and ceramide-1-phosphate, respectively. (frontiersin.org)
Phosphate6
- Sphingosine may be phosphorylated to form sphingosine-1-phosphate. (wikipedia.org)
- This leads to the formation of sphingosine-1-phosphate, a potent signaling lipid. (foodb.ca)
- SPPase, sphingosine phosphate phosphatase. (moonphase2018.com)
- The bioactive lipid sphingosine-1-phosphate (S1P) has been implicated in promoting adipogenesis, fibrosis, and inflammation in OFs. (uni-potsdam.de)
- Our previous data showed that sphingosine-1-phosphate (S1P) is released during inflammatory conditions in the lung. (unisa.it)
- Aldo-keto Reductase Family 1 Member B 10 Mediates Liver Cancer Cell Proliferation through Sphingosine-1-Phosphate. (wanfangdata.com.cn)
Lipid1
- [ 1 , 2 ] Lipid substrates share a common structure, including a ceramide backbone (2- N -acyl-sphingosine), in which various sphingolipids are derived by substitution of hexoses, phosphorylcholine, or one or more sialic acid residues on terminal hydroxyl groups of the ceramide molecule. (medscape.com)
Backbone1
- The sphingosine backbone is O-linked to a (usually) charged head group such as ethanolamine, serine, or choline. (wikipedia.org)
Fatty acids1
- For the reverse synthetic reaction, the natural sphingosine D-erythro isomer is more efficiently utilized as a substrate compared to D-erythro-dihydrosphingosine and D-erythro-phytosphingosine, while the fatty acids with chain lengths of 12 or 14 carbons are the most efficiently used (PubMed:12764132). (icr.ac.uk)
Neutral2
- Ceramidases are classified as acidic, neutral or basic according to the optimal pH with which they function. (nih.gov)
- Neutral ceramidase encoded by the Asah2 gene is essential for the intestinal degradation of sphingolipids. (musc.edu)
Enzyme1
- Most of the mutations associated with Farber lipogranulomatosis change a single protein building block (amino acid) in acid ceramidase, which severely reduces the activity of the enzyme, typically to less than one-tenth of normal. (encyclopedia.pub)
Farber1
- Acid ceramidase deficiency: Farber disease and SMA-PME. (medlink.com)
SMase1
- Together, these total outcomes demonstrate upregulation from the S-SMase/ceramide pathway in HLH, and claim that the total amount of sphingosine and ceramide determine clinical outcomes in HLH. (biobender.com)
Bases2
- The main mammalian sphingoid bases are dihydrosphingosine and sphingosine, while dihydrosphingosine and phytosphingosine are the principal sphingoid bases in yeast. (wikipedia.org)
- Sphingosine and its derivative sphinganine are the major bases of the sphingolipids in mammals. (foodb.ca)
Form2
- Finally, ceramide may be broken down by a ceramidase to form sphingosine. (wikipedia.org)
- Psychosine (Galactosyl-sphingosine) is a lyso-form of galactosyl-ceramide (GalCer), accumulated in the genetic disorder of galactosylceramidase, Krabbe's disease. (sphingolipidclub.com)
Family1
- Using pet types of chronic and severe irritation, it's been convincingly showed that plasma S1P limitations disruption of vascular endothelial monolayers and decreases oedema57.Today Sphingosine and it is family members continue to shock and confound us. (moonphase2018.com)
Cell1
- The chief advancement among these was the breakthrough of FTY720 (fingolimod), a sphingosine analogue that alters immune system cell trafficking and has already been being found in the medical clinic for the treating multiple sclerosis4. (moonphase2018.com)
Normal1
- This study provides evidence that murine acid ceramidase (Ac) is required for normal erythropoiesis and the development of rodent malaria. (elifesciences.org)
Ceramide to sphingosine1
- It plays an important role in sphingolipid degradation by catalyzing the lysosomal hydrolysis of ceramide to sphingosine and free fatty acid. (nih.gov)
Sphingolipids5
- Sphingolipids are a class of lipids containing a backbone of sphingoid bases, which are a set of aliphatic amino alcohols that includes sphingosine. (wikipedia.org)
- [ 15-17 ] Sphingolipids can be converted to a vast variety of different molecules and among these are ceramide, sphingosine, sphingomyelin and sphingosine-1-phosphate (S1P). (medscape.com)
- 6. Neutral ceramidase encoded by the Asah2 gene is essential for the intestinal degradation of sphingolipids. (nih.gov)
- Ceramide, ceramide-1-phosphate (C1P), sphingosine-1-phosphate (S1P), glycosphingolipids (particularly, gangliosides) and galactosylceramides (GalCer) are the main sphingolipid metabolites that have been considered as bioactive signaling sphingolipids in podocytes ( Figure 1 ). (scientificarchives.com)
- 1, 2] Lipid substrates share a common structure, including a ceramide backbone (2-N-acyl-sphingosine), in which various sphingolipids are derived by substitution of hexoses, phosphorylcholine, or one or more sialic acid residues on terminal hydroxyl groups of the ceramide molecule. (medscape.com)
Acid13
- The overexpressed acid ceramidase was widely discovered on cancer cells, inducing apoptotic resistance on them.125 Therefore. (immune-source.com)
- LCL521 known as an Stigmasterol (Stigmasterin) acid ceramidase inhibitor is worthy of detailed investigation. (immune-source.com)
- The ASAH1 gene provides instructions for making an enzyme called acid ceramidase. (medlineplus.gov)
- Variants in the ASAH1 gene lead to severe reduction in acid ceramidase, typically to below 10 percent of normal. (medlineplus.gov)
- It is unclear whether the level of acid ceramidase activity is related to the severity of the disorder. (medlineplus.gov)
- Alves MQ, Le Trionnaire E, Ribeiro I, Carpentier S, Harzer K, Levade T, Ribeiro MG. Molecular basis of acid ceramidase deficiency in a neonatal form of Farber disease: identification of the first large deletion in ASAH1 gene. (medlineplus.gov)
- 8. Upregulation of the human alkaline ceramidase 1 and acid ceramidase mediates calcium-induced differentiation of epidermal keratinocytes. (nih.gov)
- 15. Substrate-specificities of acid and alkaline ceramidases in fibroblasts from patients with Farber disease and controls. (nih.gov)
- These are lipid molecules containing a sphingosine in which the amine group is linked to a fatty acid. (hmdb.ca)
- AN - coordinate with organ or disease /surg HN - 2009 MH - Acid Ceramidase UI - D055573 MN - D8.811.277.87.250.100 MS - A ceramidase subtype that is active at acid pH. (nih.gov)
- Inherited deficiency of acid ceramidase activity results in FARBER LIPOGRANULOMATOSIS. (nih.gov)
- A ceramidase subtype that is active at acid pH. (nih.gov)
- Acid ceramidase (AC) is a lysosomal cysteine hydrolase that catalyzes the conversion of ceramide into fatty acid and sphingosine. (unipi.it)
Alkaline8
- 2. Role of alkaline ceramidases in the generation of sphingosine and its phosphate in erythrocytes. (nih.gov)
- 5. Alkaline ceramidase 2 is essential for the homeostasis of plasma sphingoid bases and their phosphates. (nih.gov)
- 7. Golgi alkaline ceramidase regulates cell proliferation and survival by controlling levels of sphingosine and S1P. (nih.gov)
- 10. Tumor suppressor p53 links ceramide metabolism to DNA damage response through alkaline ceramidase 2. (nih.gov)
- 14. Activity of neutral and alkaline ceramidases on fluorogenic N-acylated coumarin-containing aminodiols. (nih.gov)
- Instead of promoting expression of QS genes, ATCC 33988 up-regulates a small subset of its genome, including operons responsible for specific alkaline proteases and sphingosine metabolism. (biomedcentral.com)
- HN - 2009 MH - Alkaline Ceramidase UI - D055574 MN - D8.811.277.87.250.300 MS - A ceramidase subtype that is active at alkaline pH. (nih.gov)
- Disturbance of sphingolipid composition at the plasma membrane due to increased activity of alkaline ceramidase 2 (ACER2) increases levels of sphingosine (Sph), which, together with increased activity of sphingosine kinases 1 and 2 (SPHK1/2), leads to increased production of S1P. (scientificarchives.com)
Sphingolipid metabolism1
- 13. Role of Ceramidases in Sphingolipid Metabolism and Human Diseases. (nih.gov)
1.231
- Ceramidases (EC 3.5.1.23), such as ASAH2, catalyze hydrolysis of the N-acyl linkage of ceramide, a second messenger in a variety of cellular events, to produce sphingosine. (utsouthwestern.edu)
Metabolism1
- 9. Involvement of neutral ceramidase in ceramide metabolism at the plasma membrane and in extracellular milieu. (nih.gov)
Sphingoid bases1
- The main mammalian sphingoid bases are dihydrosphingosine and sphingosine, while dihydrosphingosine and phytosphingosine are the principal sphingoid bases in yeast. (wikipedia.org)
Hydrolysis1
- 17. Structural Basis for Ceramide Recognition and Hydrolysis by Human Neutral Ceramidase. (nih.gov)
Phytosphingosine2
- Sphingosine, dihydrosphingosine, and phytosphingosine may be phosphorylated. (wikipedia.org)
- [5] [6] Sphingosine, dihydrosphingosine, and phytosphingosine may be phosphorylated. (cloudfront.net)
Facilitates1
- We have also demonstrated that ceramidase facilitates membrane turnover and rhodopsin endocytosis in Drosophila photoreceptors. (nih.gov)
Degeneration1
- We have recently demonstrated that modulation of the sphingolipid biosynthetic pathway such as targeted expression of ceramidase, rescues degeneration in certain photoreceptor mutants. (nih.gov)
Activity1
- The ceramidase activity of ADPN receptor has been reported in many tissues: it converts ceramide into sphingosine. (bvsalud.org)