Amidohydrolases that are specific for the cleavage of the N-acyl linkage of CERAMIDES. Ceramidases are classified as acidic, neutral or basic according to the optimal pH with which they function.
A ceramidase subtype that is active at alkaline pH. It is found at high levels within the SMALL INTESTINE.
A ceramidase subtype that is active at neutral pH. It is found at high levels within the SMALL INTESTINE and in the BRAIN.
A ceramidase subtype that is active at acid pH. It plays an important role in sphingolipid degradation by catalyzing the lysosomal hydrolysis of ceramide to sphingosine and free fatty acid. Inherited deficiency of acid ceramidase activity results in FARBER LIPOGRANULOMATOSIS.
Amidohydrolases are enzymes that catalyze the hydrolysis of amides and related compounds, playing a crucial role in various biological processes including the breakdown and synthesis of bioactive molecules.
Members of the class of neutral glycosphingolipids. They are the basic units of SPHINGOLIPIDS. They are sphingoids attached via their amino groups to a long chain fatty acyl group. They abnormally accumulate in FABRY DISEASE.
An enzyme that catalyzes the hydrolysis of a ceramidetrihexoside to a ceramidedihexoside plus galactose.
An amino alcohol with a long unsaturated hydrocarbon chain. Sphingosine and its derivative sphinganine are the major bases of the sphingolipids in mammals. (Dorland, 28th ed)
Derivatives of PHOSPHATIDIC ACIDS that lack one of its fatty acyl chains due to its hydrolytic removal.

Increases in ceramide levels in normal human mesangial cells subjected to different cellular stresses result from changes in distinct enzyme activities and can influence cellular responses to other stimuli. (1/125)

Sphingolipids, ceramide in particular, have come to be regarded as having roles in cellular signaling, most recently being associated with stress and the cellular responses to stress. In the present study we first examined the mechanisms involved in the changes in cellular ceramide levels in normal human mesangial cells (NHMC) in the growth, quiescent, and senescent phases as well as those resulting from environmental stimuli. We found that in NHMC total ceramide levels increase in response to cellular stresses as a result of a combination of enzyme activities. Furthermore, different stresses cause different alterations in various enzyme activities, with age and growth influencing acidic enzymes, but cell density affecting neutral, resulting in final ceramide level increases which most likely are associated with distinct pools of ceramide. Secondly, we examined the influence of changes in ceramide levels on apoptosis induced by sphingosine and its methylated derivative N, N-dimethylsphingosine. We found that increases in cellular ceramide levels prohibited the apoptosis and caused a quiescent state in the cells. The data presented here provide additional insight into the roles of ceramide and related enzymes in cellular responses to stress and suggest a possible relevance to in vivo disease states.  (+info)

Specific and sensitive assay for alkaline and neutral ceramidases involving C12-NBD-ceramide. (2/125)

A fluorescent analogue of ceramide, C12-NBD-ceramide, was found to be hydrolyzed much faster than 14C-labeled ceramide by alkaline ceramidase from Pseudomonas aeruginosa and neutral ceramidase from mouse liver, while this substrate was relatively resistant to acid ceramidase from plasma of the horseshoe crab. The radioactive substrate was used more preferentially by the acid ceramidase. It should be noted that C6-NBD-ceramide, which is usually used for ceramidase assays, was hardly hydrolyzed by any of the enzymes examined, compared to C12-NBD-ceramide. For the alkaline and neutral enzymes, the Vmax and k (Vmax/Km) with C12-NBD-ceramide were much higher than those with 14C-ceramide. In contrast, for the acid enzyme these parameters with C12-NBD-ceramide were less than half those with the radioisotope-labeled substrate. It is noteworthy that the labeling of ceramide with NBD did not itself reduce the Km of the alkaline enzyme, but did that of the neutral enzyme. It was also found that C12-NBD-ceramide was preferentially hydrolyzed by the alkaline and neutral enzymes, but not the acid one, in several mammalian cell lines. This study clearly shows that the attachment of NBD, but not dansyl, increases the susceptibility of ceramide to alkaline and neutral enzyme, and decreases that to acid enzymes. Thus the use of this substrate provides a specific and sensitive assay for alkaline and neutral ceramidases.  (+info)

Ceramide-induced killing of normal and malignant human lymphocytes is by a non-apoptotic mechanism. (3/125)

Synthetic ceramides induce apoptotic death of Jurkat and HL60 leukaemia cell lines. By contrast we show here that ceramide induces non-apoptotic killing of malignant cells from patients with B-chronic lymphocytic leukaemia (B-CLL) and of normal B lymphocytes. The protein phosphatase inhibitor okadaic acid readily induces apoptosis of B-CLL cells, indicating that this death pathway is fully functional in these cells. The ability of ceramide to activate the apoptotic protease caspase 3 in HL60 cells but not in B-CLL cells, as well as the lack of correlation of ceramide-mediated killing of different B-CLL isolates with expression of the apoptosis-regulating proteins bcl-2 and bax reinforce the conclusion that ceramide killing of B-CLL cells is by a non-apoptotic mechanism. Fludarabine treatment or gamma-irradiation of B-CLL cells resulted in ceramide elevation and in killing by both apoptotic and non-apoptotic mechanisms, suggesting that a ceramide-triggered non-apoptotic mechanism may play a role in the killing of these cells. Therefore, the results here show that ceramide can induce either apoptotic or non-apoptotic death, depending on the cellular context. The inability of synthetic dihydroceramide to kill B-CLL cells or normal B lymphocytes suggests that non-apoptotic killing by ceramide is via interaction with a specific, but unidentified, cellular target.  (+info)

Role of sphingosine 1-phosphate in the mitogenesis induced by oxidized low density lipoprotein in smooth muscle cells via activation of sphingomyelinase, ceramidase, and sphingosine kinase. (4/125)

Oxidized LDL (oxLDL) have been implicated in diverse biological events leading to the development of atherosclerotic lesions. We previously demonstrated that the proliferation of cultured vascular smooth muscle cells (SMC) induced by oxLDL is preceded by an increase in neutral sphingomyelinase activity, sphingomyelin turnover to ceramide, and stimulation of mitogen-activated protein kinases (Auge, N., Escargueil-Blanc, I., Lajoie-Mazenc, I., Suc, I., Andrieu-Abadie, N., Pieraggi, M. T., Chatelut, M., Thiers, J. C., Jaffrezou, J. P., Laurent, G., Levade, T., Negre-Salvayre, A., and Salvayre, R. (1998) J. Biol. Chem. 273, 12893-12900). Since ceramide can be converted to other bioactive metabolites, such as the well established mitogen sphingosine 1-phosphate (S1P), we investigated whether additional ceramide metabolites are involved in the oxLDL-induced SMC proliferation. We report here that incubation of SMC with oxLDL increased the activities of both acidic and alkaline ceramidases as well as sphingosine kinase, and elevated cellular sphingosine and S1P. Furthermore, the mitogenic effect of oxLDL was inhibited by D-erythro-2-(N-myristoylamino)-1-phenyl-1-propanol and N,N-dimethylsphingosine which are inhibitors of ceramidase and sphingosine kinase, respectively. These findings suggest that S1P is a key mediator of the mitogenic effect of oxLDL. In agreement with this conclusion, exogenous addition of sphingosine stimulated the proliferation of cultured SMC, and this effect was abrogated by dimethylsphingosine but not by fumonisin B1, an inhibitor of the acylation of sphingosine to ceramide. Exogenous S1P also promoted SMC proliferation. Altogether, these results strongly suggest that the mitogenic effect of oxLDL in SMC involves the combined activation of sphingomyelinase(s), ceramidase(s), and sphingosine kinase, resulting in the turnover of sphingomyelin to a number of sphingolipid metabolites, of which at least S1P is critical for mitogenesis.  (+info)

Purification and characterization of a membrane-bound nonlysosomal ceramidase from rat brain. (5/125)

We have purified a membrane bound ceramidase 22,300-fold to apparent homogeneity. The purification scheme included Triton X-100 extraction of membranes followed by Q-Sepharose, blue Sepharose, phenyl-Sepharose, and MonoS column chromatography. The purified enzyme showed an apparent molecular mass of 90 kDa as estimated by SDS-polyacrylamide gel electrophoresis under reducing conditions and 95 kDa by chromatography on Superose 12. Using C(16)-ceramide as substrate, the enzyme showed a broad pH optimum in the neutral to alkaline range. A mixed micelle assay was developed, and using Triton X-100/ceramide mixed micelles, the enzyme exhibited classical Michaelis-Menten kinetics, with a K(m) of 1.29 mol % and a V(max) of 4.4 micromol/min/mg. When dihydroceramide was used as substrate, these values were 3.84 mol % and 1.2 micromol/min/mg, respectively, indicating that the enzyme hydrolyzes ceramides preferentially. The activity of the purified ceramidase did not require cations, and it was inhibited by reducing agents. Phosphatidylcholine and sphingomyelin were without effect on the enzyme activity, whereas phosphatidic acid and phosphatidylserine stimulated the activity 3-fold. Sphingosine acted as a competitive inhibitor with an IC(50) of 5-10 microM. These results indicate that the purified enzyme is a novel ceramidase.  (+info)

Activation of sphingosine kinase by tumor necrosis factor-alpha inhibits apoptosis in human endothelial cells. (6/125)

Human umbilical vein endothelial cells (HUVEC), like most normal cells, are resistant to tumor necrosis factor-alpha (TNF)-induced apoptosis in spite of TNF activating sphingomyelinase and generating ceramide, a known inducer of apoptosis. Here we report that TNF activates another key enzyme, sphingosine kinase (SphK), in the sphingomyelin metabolic pathway resulting in production of sphingosine-1-phosphate (S1P) and that S1P is a potent antagonist of TNF-mediated apoptosis. The TNF-induced SphK activation is independent of sphingomyelinase and ceramidase activities, suggesting that TNF affects this enzyme directly other than through a mass effect on sphingomyelin degradation. In contrast to normal HUVEC, in a spontaneously transformed endothelial cell line (C11) TNF stimulation failed to activate SphK and induced apoptosis as characterized by morphological and biochemical criteria. Addition of exogenous S1P or increasing endogenous S1P by phorbol ester markedly protected C11 cell line from TNF-induced apoptosis. Conversely, N, N-dimethylsphingosine, an inhibitor of SphK, profoundly sensitized normal HUVEC to killing by TNF. Thus, we demonstrate that the activation of SphK by TNF is an important signaling for protection from the apoptotic effect of TNF in endothelial cells.  (+info)

Molecular cloning, sequencing, and expression of the gene encoding alkaline ceramidase from Pseudomonas aeruginosa. Cloning of a ceramidase homologue from Mycobacterium tuberculosis. (7/125)

We previously reported the purification and characterization of a novel type of alkaline ceramidase from Pseudomonas aeruginosa strain AN17 (Okino, N., Tani, M., Imayama, S., and Ito, M. (1998) J. Biol. Chem. 273, 14368-14373). Here, we report the molecular cloning, sequencing, and expression of the gene encoding the ceramidase of this strain. Specific oligonucleotide primers were synthesized using the peptide sequences of the purified ceramidase obtained by digestion with lysylendopeptidase and used for polymerase chain reaction. DNA fragments thus amplified were used as probes to clone the gene encoding the ceramidase from a genomic library of strain AN17. The open reading frame of 2,010 nucleotides encoded a polypeptide of 670 amino acids including a signal sequence of 24 residues, 64 residues of which matched the amino acid sequence determined for the purified enzyme. The molecular weight of the mature enzyme was estimated to be 70,767 from the deduced amino acid sequence. Expression of the ceramidase gene in Escherichia coli, resulted in production of a soluble enzyme with the identical N-terminal amino acid sequence. Recombinant ceramidase was purified to homogeneity from the lysate of E. coli cells and confirmed to be identical to the Pseudomonas enzyme in its specificity and other enzymatic properties. No significant sequence similarities were found in other known functional proteins including human acid ceramidase. However, we found a sequence homologous to the ceramidase in hypothetical proteins encoded in Mycobacterium tuberculosis, Dictyostelium discoideum, and Arabidopsis thaliana. The homologue of the ceramidase gene was thus cloned from an M. tuberculosis cosmid and expressed in E. coli, and the gene was demonstrated to encode an alkaline ceramidase. This is the first report for the cloning of an alkaline ceramidase.  (+info)

Purification and characterization of a neutral ceramidase from mouse liver. A single protein catalyzes the reversible reaction in which ceramide is both hydrolyzed and synthesized. (8/125)

We report here a novel ceramidase that was purified more than 150, 000-fold from the membrane fraction of mouse liver. The enzyme was a monomeric polypeptide having a molecular mass of 94 kDa and was highly glycosylated with N-glycans. The amino acid sequence of a fragment obtained from the purified enzyme was homologous to those deduced from the genes encoding an alkaline ceramidase of Pseudomonas aeruginosa and a hypotheical protein of the slime mold Dictyostelium discoideum. However, no significant sequence similarities were found in other known functional proteins including acid ceramidases of humans and mice. The enzyme hydrolyzed various N-acylsphingosines but not galactosylceramide, sulfatide, GM1a, or sphingomyelin. The enzyme exhibited the highest activity around pH 7.5 and was thus identified as a type of neutral ceramidase. The apparent K(m) and V(max) values for C12-4-nitrobenzo-2-oxa-1, 3-diazole-ceramide and C16-(14)C-ceramide were 22.3 microM and 29.1 micromol/min/mg and 72.4 microM and 3.6 micromol/min/mg, respectively. This study also clearly demonstrated that the purified 94-kDa ceramidase catalyzed the condensation of fatty acid to sphingosine to generate ceramide, but did not catalyze acyl-CoA-dependent acyl-transfer reaction.  (+info)

Ceramidases are a group of enzymes that catalyze the hydrolysis of ceramide into sphingosine and free fatty acids. Ceramides are important components of cell membranes, and their metabolism is tightly regulated in cells. The hydrolysis of ceramide by ceramidases produces sphingosine, which can be further phosphorylated to form sphingosine-1-phosphate (S1P), a signaling molecule involved in various cellular processes such as proliferation, differentiation, and survival.

There are several types of ceramidases that have been identified, including acid ceramidase, neutral ceramidase, and alkaline ceramidase. These enzymes differ in their subcellular localization, substrate specificity, and physiological functions. Dysregulation of ceramidase activity has been implicated in various diseases, including cancer, neurodegenerative disorders, and inflammatory conditions. Therefore, ceramidases are considered as potential therapeutic targets for the treatment of these diseases.

Alkaline ceramidase is a type of enzyme that belongs to the family of hydrolases, specifically those acting on ester bonds. This enzyme's function is to catalyze the hydrolysis of ceramides into sphingosine and free fatty acids. Ceramides are important lipid molecules found in cell membranes, and their metabolism plays a crucial role in various biological processes such as cell differentiation, proliferation, and apoptosis.

Alkaline ceramidase is localized in the endoplasmic reticulum and Golgi apparatus of cells and has an optimum pH range between 8.5 to 9.5. It is involved in several physiological processes, including skin barrier formation, inflammation, and cancer development. Mutations in the gene that encodes for alkaline ceramidase have been associated with various diseases such as Farber's lipogranulomatosis, a rare genetic disorder characterized by accumulation of ceramides in tissues leading to joint pain, hoarseness, and progressive intellectual disability.

Neutral ceramidase is an enzyme that plays a role in the metabolism of sphingolipids, which are a type of lipid found in cell membranes. Specifically, neutral ceramidase catalyzes the conversion of ceramide to sphingosine and free fatty acid. This reaction takes place at a neutral pH, hence the name "neutral" ceramidase.

Ceramide is a key component of the lipid bilayer in cell membranes and is also involved in various signaling pathways related to cell growth, differentiation, and apoptosis (programmed cell death). The conversion of ceramide to sphingosine by neutral ceramidase helps to regulate these processes.

Abnormal levels or activity of neutral ceramidase have been implicated in various diseases, including cancer, inflammation, and neurodegenerative disorders. For example, increased activity of this enzyme has been observed in some types of cancer, which may contribute to tumor growth and progression. On the other hand, decreased activity of neutral ceramidase has been linked to inflammatory conditions and neurodegenerative diseases such as Alzheimer's disease.

Acid ceramidase is an enzyme that plays a role in the metabolism of ceramides, which are lipid molecules found in cell membranes. Specifically, acid ceramidase helps to break down ceramides into sphingosine and free fatty acids. This enzyme is active at an acidic pH and is located in the lysosomes, which are organelles within cells that help to break down and recycle various materials.

Defects in the gene that provides instructions for making acid ceramidase can lead to a condition called Farber disease, which is characterized by the accumulation of ceramides in various tissues and organs. This can cause a range of symptoms, including joint pain, muscle weakness, and developmental delays.

Amidohydrolases are a class of enzymes that catalyze the hydrolysis of amides and related compounds, resulting in the formation of an acid and an alcohol. This reaction is also known as amide hydrolysis or amide bond cleavage. Amidohydrolases play important roles in various biological processes, including the metabolism of xenobiotics (foreign substances) and endogenous compounds (those naturally produced within an organism).

The term "amidohydrolase" is a broad one that encompasses several specific types of enzymes, such as proteases, esterases, lipases, and nitrilases. These enzymes have different substrate specificities and catalytic mechanisms but share the common ability to hydrolyze amide bonds.

Proteases, for example, are a major group of amidohydrolases that specifically cleave peptide bonds in proteins. They are involved in various physiological processes, such as protein degradation, digestion, and regulation of biological pathways. Esterases and lipases hydrolyze ester bonds in various substrates, including lipids and other organic compounds. Nitrilases convert nitriles into carboxylic acids and ammonia by cleaving the nitrile bond (C≡N) through hydrolysis.

Amidohydrolases are found in various organisms, from bacteria to humans, and have diverse applications in industry, agriculture, and medicine. For instance, they can be used for the production of pharmaceuticals, biofuels, detergents, and other chemicals. Additionally, inhibitors of amidohydrolases can serve as therapeutic agents for treating various diseases, such as cancer, viral infections, and neurodegenerative disorders.

Ceramides are a type of lipid molecule that are found naturally in the outer layer of the skin (the stratum corneum). They play a crucial role in maintaining the barrier function and hydration of the skin. Ceramides help to seal in moisture, support the structure of the skin, and protect against environmental stressors such as pollution and bacteria.

In addition to their role in the skin, ceramides have also been studied for their potential therapeutic benefits in various medical conditions. For example, abnormal levels of ceramides have been implicated in several diseases, including diabetes, cardiovascular disease, and cancer. As a result, ceramide-based therapies are being investigated as potential treatments for these conditions.

Medically, ceramides may be mentioned in the context of skin disorders or diseases where there is a disruption in the skin's barrier function, such as eczema, psoriasis, and ichthyosis. In these cases, ceramide-based therapies may be used to help restore the skin's natural barrier and improve its overall health and appearance.

Galactosylgalactosylglucosylceramidase is a type of enzyme that is involved in the breakdown and recycling of complex lipids called glycosphingolipids in the body. More specifically, it helps to break down a particular type of glycosphingolipid known as globotriaosylceramide (Gb3 or CD77) into simpler components.

This enzyme is critical for maintaining the health and function of various tissues in the body, including the nervous system. Deficiencies in galactosylgalactosylglucosylceramidase have been linked to a number of serious genetic disorders, such as Tay-Sachs disease and Sandhoff disease, which are characterized by the accumulation of Gb3 and other glycosphingolipids in various tissues, leading to progressive neurological deterioration and other symptoms.

Sphingosine is not a medical term per se, but rather a biological compound with importance in the field of medicine. It is a type of sphingolipid, a class of lipids that are crucial components of cell membranes. Sphingosine itself is a secondary alcohol with an amino group and two long-chain hydrocarbons.

Medically, sphingosine is significant due to its role as a precursor in the synthesis of other sphingolipids, such as ceramides, sphingomyelins, and gangliosides, which are involved in various cellular processes like signal transduction, cell growth, differentiation, and apoptosis (programmed cell death).

Moreover, sphingosine-1-phosphate (S1P), a derivative of sphingosine, is an important bioactive lipid mediator that regulates various physiological functions, including immune response, vascular maturation, and neuronal development. Dysregulation of S1P signaling has been implicated in several diseases, such as cancer, inflammation, and cardiovascular disorders.

In summary, sphingosine is a crucial biological compound with medical relevance due to its role as a precursor for various sphingolipids involved in cellular processes and as a precursor for the bioactive lipid mediator S1P.

Lysophospholipids are a type of glycerophospholipid, which is a major component of cell membranes. They are characterized by having only one fatty acid chain attached to the glycerol backbone, as opposed to two in regular phospholipids. This results in a more polar and charged molecule, which can play important roles in cell signaling and regulation.

Lysophospholipids can be derived from the breakdown of regular phospholipids through the action of enzymes such as phospholipase A1 or A2. They can also be synthesized de novo in the cell. Some lysophospholipids, such as lysophosphatidic acid (LPA) and sphingosine-1-phosphate (S1P), have been found to act as signaling molecules that bind to specific G protein-coupled receptors and regulate various cellular processes, including proliferation, survival, and migration.

Abnormal levels of lysophospholipids have been implicated in several diseases, such as cancer, inflammation, and neurological disorders. Therefore, understanding the biology of lysophospholipids has important implications for developing new therapeutic strategies.

Presently, 7 human ceramidases encoded by 7 distinct genes have been cloned: acid ceramidase (ASAH1) - cell survival neutral ... Ceramidase at the U.S. National Library of Medicine Medical Subject Headings (MeSH) EC 3.5.1.23 Portal: Biology v t e (EC 3.5.1 ... Human neutral ceramidase (nCDase) is an enzyme that plays a critical role in colon cancer and there are currently no potent or ... Ceramidase (EC 3.5.1.23, acylsphingosine deacylase, glycosphingolipid ceramide deacylase) is an enzyme which cleaves fatty ...
The ASAH1 gene codes for acid ceramidase, an enzyme found in lysosomes. The lysosome breaks down acid ceramidase; the fatty ... In patients with SMA-PME, the ceramidase function decreases to 33.33% effective. The lack of myelin resulting from the lack of ... Park, Jae-Ho; Schuchman, Edward H. (December 2006). "Acid ceramidase and human disease". Biochimica et Biophysica Acta (BBA) - ... "Acid ceramidase deficiency associated with spinal muscular atrophy with progressive myoclonic epilepsy". Neuromuscular ...
Okino N, He X, Gatt S, Sandhoff K, Ito M, Schuchman EH (August 2003). "The reverse activity of human acid ceramidase". The ... Seelan RS, Qian C, Yokomizo A, Bostwick DG, Smith DI, Liu W (October 2000). "Human acid ceramidase is overexpressed but not ... Bär J, Linke T, Ferlinz K, Neumann U, Schuchman EH, Sandhoff K (March 2001). "Molecular analysis of acid ceramidase deficiency ... The ASAH1 gene encodes in humans the acid ceramidase enzyme. This gene encodes a heterodimeric protein consisting of a ...
Neutral ceramidase is an enzyme that in humans is encoded by the ASAH2 gene. GRCh38: Ensembl release 89: ENSG00000188611 - ... 2004). "Neutral ceramidase gene: role in regulating ceramide-induced apoptosis". Gene. 315: 113-22. doi:10.1016/S0378-1119(03) ... 2006). "Golgi alkaline ceramidase regulates cell proliferation and survival by controlling levels of sphingosine and S1P". ... Overview of all the structural information available in the PDB for UniProt: Q9NR71 (Neutral ceramidase; ASAH2) at the PDBe-KB ...
... autosomal recessive lysosomal storage disease caused by a deficiency of the acid ceramidase enzyme. Acid ceramidase is ... This gene codes for the acid ceramidase enzyme. Individuals with Farber disease have two copies of this gene that are not ... ISBN 978-0-7216-2921-6. Yu, FPS; Amintas, S; Levade, T; Medin, JA (20 July 2018). "Acid ceramidase deficiency: Farber disease ... Farber disease (also known as Farber's lipogranulomatosis, acid ceramidase deficiency, "Lipogranulomatosis", and ASAH1-related ...
Alkaline ceramidase 3 also known as ACER3 is a ceramidase enzyme which in humans is encoded by the ACER3 gene. GRCh38: Ensembl ... Mao C, Obeid LM (September 2008). "Ceramidases: regulators of cellular responses mediated by ceramide, sphingosine, and ... "Cloning and characterization of a novel human alkaline ceramidase. A mammalian enzyme that hydrolyzes phytoceramide". J. Biol. ...
Neutral ceramidase C also known as N-acylsphingosine amidohydrolase 2C or non-lysosomal ceramidase C or ASAH2C is a ceramidase ... Hwang YH, Tani M, Nakagawa T, Okino N, Ito M (May 2005). "Subcellular localization of human neutral ceramidase expressed in ... Choi MS, Anderson MA, Zhang Z, Zimonjic DB, Popescu N, Mukherjee AB (October 2003). "Neutral ceramidase gene: role in ... "Identification of a novel amidase motif in neutral ceramidase". The Biochemical Journal. 393 (Pt 3): 687-95. doi:10.1042/ ...
2005). "Elevation of the level and activity of acid ceramidase in Alzheimer's disease brain". Eur. J. Neurosci. 20 (12): 3489- ... This gene encodes an N-acylethanolamine-hydrolyzing enzyme which is highly similar to acid ceramidase. Multiple transcript ... "Entrez Gene: ASAHL N-acylsphingosine amidohydrolase (acid ceramidase)-like". Human NAAA genome location and NAAA gene details ... Schulze H, Schepers U, Sandhoff K (2008). "Overexpression and mass spectrometry analysis of mature human acid ceramidase". Biol ...
Alkaline ceramidase 2 also known as ACER2 is a ceramidase enzyme which in humans is encoded by the ACER2 gene. The ACER2/ ... Sun W, Hu W, Xu R, Jin J, Szulc ZM, Zhang G, Galadari SH, Obeid LM, Mao C (February 2009). "Alkaline ceramidase 2 regulates ... 2006). "Golgi alkaline ceramidase regulates cell proliferation and survival by controlling levels of sphingosine and S1P". ... Mao C, Obeid LM (September 2008). "Ceramidases: regulators of cellular responses mediated by ceramide, sphingosine, and ...
Sphingosine (Sph) is formed by the action of ceramidase (CDase) enzymes on ceramide in the lysosome. Sph can also be formed in ... The low levels of Sph and their increase in response to stimulation of cells, primarily by activation of ceramidase by growth- ... 2006). "Golgi alkaline ceramidase regulates cell proliferation and survival by controlling levels of sphingosine and S1P". ... Ceramide can also be broken down by enzymes called ceramidases, leading to the formation of sphingosine, Moreover, a phosphate ...
December 2017). "Acid ceramidase and its inhibitors: a de novo drug target and a new class of drugs for killing glioblastoma ... April 2017). "Acid ceramidase is a novel drug target for pediatric brain tumors". Oncotarget. 8 (15): 24753-24761. doi:10.18632 ... However, carmofur is a highly potent acid ceramidase (AC) inhibitor. Ceramide influences cancer cell survival, growth and death ... Jan 2013). "Discovery of highly potent acid ceramidase inhibitors with in vitro tumor chemosensitizing activity". Scientific ...
Alkaline ceramidase 1 also known as ACER1 is a ceramidase enzyme which in humans is encoded by the ACER1 gene. ACER1 mediates ... "Upregulation of the human alkaline ceramidase 1 and acid ceramidase mediates calcium-induced differentiation of epidermal ... Ito M, Okino N, Tani M, Mitsutake S, Mori K (Mar 2002). "[Molecular evolution of neutral ceramidase: signalling molecule and ... "Cloning and characterization of a mouse endoplasmic reticulum alkaline ceramidase: an enzyme that preferentially regulates ...
In chronic myelogenous leukemia (CML), IRF8 regulates acid ceramidase to mediate CML apoptosis. IRF8 is highly expressed in ... "IRF8 regulates acid ceramidase expression to mediate apoptosis and suppresses myelogeneous leukemia". Cancer Res. 71 (8): 2882- ...
Finally, ceramide may be broken down by a ceramidase to form sphingosine. Sphingosine may be phosphorylated to form sphingosine ...
Ceramidase, an enzyme primarily present in plasma membrane, will convert ceramide to sphingosine. sphingosine is then ...
... neutral ceramidase, and sphingolipid metabolites". Pediatr. Res. 61 (1): 61-6. doi:10.1203/01.pdr.0000250534.92934.c2. PMID ...
"A neutral ceramidase, NlnCDase, is involved in the stress responses of brown planthopper, Nilaparvata lugens (Stål)". ...
... de formation via de novo sphinganine and fatty acyl-CoA and via sphingosine produced by the breakdown of ceramide by ceramidase ...
On the other hand, NAAA is homologous to acid ceramidase (HUGO gene symbol: ASAH1), sharing 30% sequence identity at the amino ... NAAA's primary structure is nearly identical to that of acid ceramidase (AC), whose only difference is the substitution of Leu ... While it was discovered within the last decade, its structural similarity to the more familiar acid ceramidase (AC) and ... a novel member of the choloylglycine hydrolase family with structural and functional similarity to acid ceramidase". The ...
... can be further hydrolyzed by acid ceramidase to form sphingosine and a free fatty acid, both of which are able to ... Anandamide Ceramidase inhibitors Chemotherapeutic agents Fas ligand Endotoxin Homocysteine Heat Hydroxypalmitoyl Sphinganine ...
... and they identified acid ceramidase as the enzyme deficient in Farber's disease. In 1964, Moser returned to the Joseph P. ...
Ace (disambiguation) Ace 2 (disambiguation) acel (disambiguation) acei (disambiguation) ACER1 (alkaline ceramidase 1) This ...
Neutral ceramidase B also known as non-lysosomal ceramidase B or N-acylsphingosine amidohydrolase (non-lysosomal ceramidase) 2B ... or ASAH2B is a ceramidase enzyme which in humans is encoded by the ASAH2B gene. ASAH2B shows reduced expression with increasing ...
... instability immunodeficiency syndrome Centronuclear myopathy Centrotemporal epilepsy Cephalopolysyndactyly Ceramidase ...
Acid Ceramidase inhibitors). Daniele Piomelli is scientific cofounder of Kadmus Pharmaceuticals and Thesan Pharmaceuticals. Di ... "Discovery of highly potent acid ceramidase inhibitors with in vitro tumor chemosensitizing activity". Science Reports 3:1035. ...
... acid ceramidase), an enzymatic product of ASAH1, gene mutated in Farber disease Australian Society for Asian Humanities This ...
... acid ceramidase) 1 ASPH: encoding enzyme Aspartyl/asparaginyl beta-hydroxylase AZIN1: encoding protein Antizyme inhibitor 1 ...
... s include: Sphingolipidoses Ceramidase Farber disease Krabbe disease Infantile onset Late onset ...
It is an inhibitor of the sphingolipid signaling pathway, via specific ceramidase inhibition (ceramidase converts ceramide to ...
... ceramidase EC 3.5.1.24: choloylglycine hydrolase EC 3.5.1.25: N-acetylglucosamine-6-phosphate deacetylase EC 3.5.1.26: N4-(β-N- ...
In humans, five different ceramidases are known-acid ceramidase, neutral ceramidase, and alkaline ceramidase 1, 2, and 3-which ... A) Acid ceramidase (PDB ID: 6MHM); (B) neutral ceramidase (PDB ID: 4WGK); (C) alkaline ceramidase (PDB ID: 6G7O); and (D) the ... Role of Ceramidases in Sphingolipid Metabolism and Human Diseases Farzana Parveen 1 , Daniel Bender 2 , Shi-Hui Law 1 , Vineet ... Role of Ceramidases in Sphingolipid Metabolism and Human Diseases Farzana Parveen et al. Cells. 2019. . ...
... a cellular ceramidase activity that catalyses the hydrolysis of ceramide to produce sphingosine and a free fatty acid (FFA). ... Structural insights into adiponectin receptors suggest ceramidase activity Ieva Vasiliauskaité-Brooks 1 , Remy Sounier 1 , ... Structural insights into adiponectin receptors suggest ceramidase activity Ieva Vasiliauskaité-Brooks et al. Nature. 2017. . ... These data strongly suggest that all members of the PAQR family may have a ceramidase activity. (c) Sequence alignment of the ...
Purchase Recombinant Alkaline ceramidase (W02F12.2). It is produced in in vitro E.coli expression system. High purity. Good ... Recombinant Alkaline ceramidase (W02F12.2), partial ( Yeast-CSB-YP524292CXY1 E.coli-CSB-EP524292CXY1 Baculovirus-CSB- ...
PROGESTIN AND ADIPOQ RECEPTOR FAMILY, integral membrane protein, 7TM, ceramidase, membrane protein ...
Role for myeloid acid ceramidase in colon inflammation and cancer. *Snider, Ashley J (PI) ...
"The enzyme ceramidase is a new, completely unexpected target structure for antiviral therapy," says Professor Jochen Bodem from ... The enzyme ceramidase inhibition by Fluoxetine emerges as a new potential therapy of Covid-19. ... The enzyme ceramidase inhibition by Fluoxetine emerges as a new potential therapy of Covid-19 ... In the lysosomes, both active substances also suppress the activity of an enzyme group, the acid ceramidases. This increases ...
Acid ceramidase. [13] AT1B1_HUMAN. Sodium/potassium-transporting ATPase subunit-β1 [13] ...
Acid ceramidase breaks down fats called ceramides into a fat called sphingosine and a fatty acid. These two breakdown products ... Acid ceramidase deficiency: Farber disease and SMA-PME. Orphanet J Rare Dis. 2018 Jul 20;13(1):121. doi: 10.1186/s13023-018- ... Variants in the ASAH1 gene lead to severe reduction in acid ceramidase, typically to below 10 percent of normal. As a result, ... The ASAH1 gene provides instructions for making an enzyme called acid ceramidase. This enzyme is found in cell compartments ...
Ceramidases (CDase) are enzymes that catalyze the hydrolysis of N-acyl linkage of ceramide (Cer) to generate sphingosine and ... N2 - Ceramidases (CDase) are enzymes that catalyze the hydrolysis of N-acyl linkage of ceramide (Cer) to generate sphingosine ... AB - Ceramidases (CDase) are enzymes that catalyze the hydrolysis of N-acyl linkage of ceramide (Cer) to generate sphingosine ... abstract = "Ceramidases (CDase) are enzymes that catalyze the hydrolysis of N-acyl linkage of ceramide (Cer) to generate ...
The maintenance of diminished acid ceramidase (ASAH1) gene expression leading to the accumulation of antiproliferative ... Altogether, these data indicated that PEDHC produced by P. gingivalis inhibits acid ceramidase expression, promotes ... Inhibitory effect of Porphyromonas gingivalis-derived phosphoethanolamine dihydroceramide on acid ceramidase expression in oral ... Ceramidase Ácida/genética Estudos Prospectivos Células Epiteliais/metabolismo Ceramidas Carcinoma de Células Escamosas de ...
Also known as: Asah2tm1Rlp, neutral ceramidase KO mouse. Asah2 KO mice lack expression of neutral ceramidase, which is highly ... Asah2 KO mice are viable and grossly normal, lack expression of neutral ceramidase and… ...
Abbreviations: ACER2, alkaline ceramidase 2; FUT4, fucosyltransferase 4; GCNT1, glucosaminyl (N-acetyl) transferase 1, core 2; ...
Farbers disease is caused by a deficiency of the enzyme called ceramidase. It affects both males and females. Disease onset is ...
Acid ceramidase deficiency, see Farber lipogranulomatosis. *Acid esterase deficiency, see Lysosomal acid lipase deficiency ...
In vivo delivery of human acid ceramidase via cord blood transplantation and direct injection of lentivirus as novel treatment ... Ceramidase is encoded by the gene ASAH localized to chromosome 8p22-p21.3. ... This autosomal recessive disorder results from deficiency of lysosomal enzyme, ceramidase, and accumulation of ceramide in ...
Alkaline ceramidase 2 regulates beta1 integrin maturation and cell adhesion.. Sun WHu WXu RJin JSzulc ZMZhang GGaladari SHObeid ... Correction: Alkaline Ceramidase 3 Deficiency Results in Purkinje Cell Degeneration and Cerebellar Ataxia Due to Dyshomeostasis ... Alkaline Ceramidase 3 Deficiency Results in Purkinje Cell Degeneration and Cerebellar Ataxia Due to Dyshomeostasis of ...
Acid ceramidase deficiency; Farber lipogranulomatosisAcid ceramidase deficiency; Farber lipogranulomatosis. Read More ...
MeSH Terms: Acid Ceramidase/genetics; Algorithms; Artificial Intelligence; Chromosomes, Human, Pair 10/genetics; Family*; Genes ...
Role of Ceramidases in Sphingolipid Metabolism and Human Diseases.. Parveen F; Bender D; Law SH; Mishra VK; Chen CC; Ke LY. ... Alkaline ceramidase 2 is essential for the homeostasis of plasma sphingoid bases and their phosphates.. Li F; Xu R; Low BE; Lin ... Ceramidases, roles in sphingolipid metabolism and in health and disease.. Coant N; Sakamoto W; Mao C; Hannun YA. Adv Biol Regul ... 4. Role of alkaline ceramidases in the generation of sphingosine and its phosphate in erythrocytes.. Xu R; Sun W; Jin J; Obeid ...
Receptor-mediated activation of ceramidase activity initiates the pleiotropic actions of adiponectin. Nature Medicine, 2011 Jan ...
A buildup of ceramides in the retina, likely due to insufficient ceramidase activity, led to photoreceptor death. When treated ...
Farbers disease is caused by a deficiency of the enzyme called ceramidase. It affects both males and females. Disease onset is ...
Ceramidases Preferred Term Term UI T714763. Date02/21/2008. LexicalTag NON. ThesaurusID NLM (2009). ... Ceramidases Preferred Concept UI. M0518596. Registry Number. EC 3.5.1.23. Scope Note. Amidohydrolases that are specific for the ... Ceramidase Term UI T714911. Date02/22/2008. LexicalTag NON. ThesaurusID NLM (2009). ... Ceramidases are classified as acidic, neutral or basic according to the optimal pH with which they function.. Entry Term(s). ...
Ceramidases Preferred Term Term UI T714763. Date02/21/2008. LexicalTag NON. ThesaurusID NLM (2009). ... Ceramidases Preferred Concept UI. M0518596. Registry Number. EC 3.5.1.23. Scope Note. Amidohydrolases that are specific for the ... Ceramidase Term UI T714911. Date02/22/2008. LexicalTag NON. ThesaurusID NLM (2009). ... Ceramidases are classified as acidic, neutral or basic according to the optimal pH with which they function.. Entry Term(s). ...
Acid ceramidase regulates innate immune memory. Rother, N., Yanginlar, C., Prévot, G., Jonkman, I., Jacobs, M., van Leent, M. M ...
Farber disease, disseminated lipogranulomatosis (ceramidase deficiency): Farber disease starts to manifest in infancy as a ...
Ceramidase (substance) {259437003 , SNOMED-CT } Parent/Child (Relationship Type) Galactosylceramidase (substance) {6590001 , ...
Ceramidase, Glucosyl Glucocerebrosidase Glucocerebroside beta Glucosidase Glucocerebroside beta-Glucosidase Glucosyl Ceramidase ... Ceramidase, Glucosyl. Glucocerebrosidase. Glucocerebroside beta Glucosidase. Glucocerebroside beta-Glucosidase. Glucosyl ... Ceramidase. Glucosyl Hydrolase, Glucosylsphingosine. Glucosylceramide beta Glucosidase. Glucosylceramide beta-Glucosidase. ...
  • Presently, 7 human ceramidases encoded by 7 distinct genes have been cloned: acid ceramidase (ASAH1) - cell survival neutral ceramidase (ASAH2, ASAH2B, ASAH2C) - protective against inflammatory cytokines alkaline ceramidase 1 (ACER1) - mediating cell differentiation by controlling the generation of SPH and S1P alkaline ceramidase 2 (ACER2) - important for cell proliferation and survival alkaline ceramidase 3 (ACER3) A deficiency in ASAH1 is associated with Farber disease. (wikipedia.org)
  • The ASAH1 gene provides instructions for making an enzyme called acid ceramidase. (medlineplus.gov)
  • Acid ceramidase breaks down fats called ceramides into a fat called sphingosine and a fatty acid. (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)
  • Inhibitory effect of Porphyromonas gingivalis-derived phosphoethanolamine dihydroceramide on acid ceramidase expression in oral squamous cells. (bvsalud.org)
  • The maintenance of diminished acid ceramidase (ASAH1) gene expression leading to the accumulation of antiproliferative intracellular ceramides in oral squamous cell carcinoma (OSCC) has emerged as a prospective oral cancer therapeutic regimen. (bvsalud.org)
  • Altogether, these data indicated that PEDHC produced by P. gingivalis inhibits acid ceramidase expression, promotes intracellular ceramide accumulation and suppresses the survival and migration of OSCC cells in vitro . (bvsalud.org)
  • Acid ceramidase involved in pathogenic cascade leading to accumulation of a-synuclein in iPSC model of GBA1-associated Parkinson's disease. (harvard.edu)
  • Oleylethanolamide is an inhibitor of Acid Ceramidase and an activator of PPARα and VR1. (geroprotectors.org)
  • This leads to accumulation of cholesterol in lysosomes, which is sustained by decreased activity of acid ceramidase (ACDase). (encyclopedia.pub)
  • Lysosomal acid ceramidase (Ac) has been shown to be critical for ceramide hydrolysis and regulation of lysosome function and cellular homeostasis. (lab.equipment)
  • Ceramidase (EC 3.5.1.23, acylsphingosine deacylase, glycosphingolipid ceramide deacylase) is an enzyme which cleaves fatty acids from ceramide, producing sphingosine (SPH) which in turn is phosphorylated by a sphingosine kinase to form sphingosine-1-phosphate (S1P). (wikipedia.org)
  • Sphingosine is produced in the sphingolipid metabolic pathway via the interaction between ceramide and ceramidase. (avantilipids.com)
  • Human neutral ceramidase (nCDase) is an enzyme that plays a critical role in colon cancer and there are currently no potent or clinically effective inhibitors for nCDase reported to date. (wikipedia.org)
  • Completely unexpectedly, the enzyme ceramidase emerges as a new target structure for the therapy of SARS-CoV-2 infections. (uni-wuerzburg.de)
  • N-Metallocenoylsphingosines as targeted ceramidase inhibitors : Syntheses and antitumoral effects. (uni-bayreuth.de)
  • AdipoR1 and AdipoR2 (AdipoRs) are plasma membrane proteins often considered to act as adiponectin receptors with a ceramidase activity. (biomedcentral.com)
  • Acid ceramidase breaks down fats called ceramides into a fat called sphingosine and a fatty acid. (medlineplus.gov)
  • 10. Ceramidases, roles in sphingolipid metabolism and in health and disease. (nih.gov)
  • 4. Role of alkaline ceramidases in the generation of sphingosine and its phosphate in erythrocytes. (nih.gov)
  • 12. Deficiency of the alkaline ceramidase ACER3 manifests in early childhood by progressive leukodystrophy. (nih.gov)
  • 16. Alkaline ceramidase 2 is essential for the homeostasis of plasma sphingoid bases and their phosphates. (nih.gov)
  • A buildup of ceramides in the retina, likely due to insufficient ceramidase activity, led to photoreceptor death. (nih.gov)
  • In this study we report the purification and characterization of a novel second type of neutral ceramidase from rat brain (RBCDase II). (nyu.edu)
  • Ceramidases are classified as acidic, neutral or basic according to the optimal pH with which they function. (nih.gov)