Neutral Ceramidase: A ceramidase subtype that is active at neutral pH. It is found at high levels within the SMALL INTESTINE and in the BRAIN.Acid Ceramidase: 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.Alkaline Ceramidase: A ceramidase subtype that is active at alkaline pH. It is found at high levels within the SMALL INTESTINE.Ceramidases: 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.Galactosylgalactosylglucosylceramidase: An enzyme that catalyzes the hydrolysis of a ceramidetrihexoside to a ceramidedihexoside plus galactose.AmidohydrolasesCeramides: 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.Sphingosine: 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)Sphingomyelin Phosphodiesterase: An enzyme that catalyzes the hydrolysis of sphingomyelin to ceramide (N-acylsphingosine) plus choline phosphate. A defect in this enzyme leads to NIEMANN-PICK DISEASE. EC 3.1.4.12.Myristates: Salts and esters of the 14-carbon saturated monocarboxylic acid--myristic acid.Sphingolipids: A class of membrane lipids that have a polar head and two nonpolar tails. They are composed of one molecule of the long-chain amino alcohol sphingosine (4-sphingenine) or one of its derivatives, one molecule of a long-chain acid, a polar head alcohol and sometimes phosphoric acid in diester linkage at the polar head group. (Lehninger et al, Principles of Biochemistry, 2nd ed)Sphingomyelins: A class of sphingolipids found largely in the brain and other nervous tissue. They contain phosphocholine or phosphoethanolamine as their polar head group so therefore are the only sphingolipids classified as PHOSPHOLIPIDS.Lysophospholipids: Derivatives of PHOSPHATIDIC ACIDS that lack one of its fatty acyl chains due to its hydrolytic removal.Peptaibols: A group of peptides characterized by length of 1-2 dozen residues with a high proportion of them being non-proteinogenic, notably alpha-aminoisobutyric acid (Aib) and isovaline, and have a C-terminal amino alcohol and N terminal alkyl group. They are found in FUNGI and some are ANTI-INFECTIVE AGENTS. They form channels or pores in target organisms. The term is a contraction of peptide-Aib-alcohol.Lysosomal Storage Diseases: Inborn errors of metabolism characterized by defects in specific lysosomal hydrolases and resulting in intracellular accumulation of unmetabolized substrates.Sphingolipidoses: A group of inherited metabolic disorders characterized by the intralysosomal accumulation of SPHINGOLIPIDS primarily in the CENTRAL NERVOUS SYSTEM and to a variable degree in the visceral organs. They are classified by the enzyme defect in the degradation pathway and the substrate accumulation (or storage). Clinical features vary in subtypes but neurodegeneration is a common sign.Sphingolipid Activator Proteins: A family of glycoprotein cofactors that are required for the efficient catabolization of SPHINGOLIPIDS by specific acid hydrolases such as GLUCOSYLCERAMIDASE; GALACTOCEREBROSIDASE; BETA-N-ACETYLHEXOSAMINIDASE; and CEREBROSIDE-SULFATASE.Serine C-Palmitoyltransferase: A key enzyme in SPHINGOLIPIDS biosynthesis, this enzyme catalyzes the pyridoxal-5'-phosphate-dependent condensation of L-SERINE and PALMITOYL COENZYME A to 3-dehydro-D-sphinganine. The enzyme consists of two different subunits.Monoglycerides: GLYCEROL esterified with a single acyl (FATTY ACIDS) chain.Fumonisins: A group of MYCOTOXINS found in CORN contaminated with FUSARIUM fungus. They are chains of about 20 carbons with acidic ester, acetylamino and sometimes other substituents. They inhibit ceramide synthetase conversion of SPHINGOLIPIDS to CERAMIDES.Hydrolysis: The process of cleaving a chemical compound by the addition of a molecule of water.Ethanolamines: AMINO ALCOHOLS containing the ETHANOLAMINE; (-NH2CH2CHOH) group and its derivatives.Oleic Acids: A group of fatty acids that contain 18 carbon atoms and a double bond at the omega 9 carbon.Substrate Specificity: A characteristic feature of enzyme activity in relation to the kind of substrate on which the enzyme or catalytic molecule reacts.Propanolamines: AMINO ALCOHOLS containing the propanolamine (NH2CH2CHOHCH2) group and its derivatives.Hydrogen-Ion Concentration: The normality of a solution with respect to HYDROGEN ions; H+. It is related to acidity measurements in most cases by pH = log 1/2[1/(H+)], where (H+) is the hydrogen ion concentration in gram equivalents per liter of solution. (McGraw-Hill Dictionary of Scientific and Technical Terms, 6th ed)Glomerular Mesangium: The thin membranous structure supporting the adjoining glomerular capillaries. It is composed of GLOMERULAR MESANGIAL CELLS and their EXTRACELLULAR MATRIX.Glucosylceramides: Cerebrosides which contain as their polar head group a glucose moiety bound in glycosidic linkage to the hydroxyl group of ceramides. Their accumulation in tissue, due to a defect in beta-glucosidase, is the cause of Gaucher's disease.Phosphotransferases (Alcohol Group Acceptor): A group of enzymes that transfers a phosphate group onto an alcohol group acceptor. EC 2.7.1.Endocannabinoids: Fatty acid derivatives that have specificity for CANNABINOID RECEPTORS. They are structurally distinct from CANNABINOIDS and were originally discovered as a group of endogenous CANNABINOID RECEPTOR AGONISTS.Dolichol Phosphates: Phosphoric acid esters of dolichol.Saposins: A group of four homologous sphingolipid activator proteins that are formed from proteolytic cleavage of a common protein precursor molecule referred to as prosaposin.Electronic Mail: Messages between computer users via COMPUTER COMMUNICATION NETWORKS. This feature duplicates most of the features of paper mail, such as forwarding, multiple copies, and attachments of images and other file types, but with a speed advantage. The term also refers to an individual message sent in this way.Farber Lipogranulomatosis: A sphingolipidosis subtype that is characterized by the histological appearance of granulomatous deposits in tissues. It results from the accumulation of CERAMIDES in various tissues due to an inherited deficiency of ACID CERAMIDASE.Energy Metabolism: The chemical reactions involved in the production and utilization of various forms of energy in cells.Epidermis: The external, nonvascular layer of the skin. It is made up, from within outward, of five layers of EPITHELIUM: (1) basal layer (stratum basale epidermidis); (2) spinous layer (stratum spinosum epidermidis); (3) granular layer (stratum granulosum epidermidis); (4) clear layer (stratum lucidum epidermidis); and (5) horny layer (stratum corneum epidermidis).Receptors, Adiponectin: Cell surface receptors for ADIPONECTIN, an antidiabetic hormone secreted by ADIPOCYTES. Adiponectin receptors are membrane proteins with multiple cytoplasmic and extracellular regions. They are about 43 kDa and encoded by at least two genes with different affinities for globular and full-length adiponectin.ArchivesBiological Science Disciplines: All of the divisions of the natural sciences dealing with the various aspects of the phenomena of life and vital processes. The concept includes anatomy and physiology, biochemistry and biophysics, and the biology of animals, plants, and microorganisms. It should be differentiated from BIOLOGY, one of its subdivisions, concerned specifically with the origin and life processes of living organisms.Periodicals as Topic: A publication issued at stated, more or less regular, intervals.Breast Neoplasms: Tumors or cancer of the human BREAST.Apoptosis: One of the mechanisms by which CELL DEATH occurs (compare with NECROSIS and AUTOPHAGOCYTOSIS). Apoptosis is the mechanism responsible for the physiological deletion of cells and appears to be intrinsically programmed. It is characterized by distinctive morphologic changes in the nucleus and cytoplasm, chromatin cleavage at regularly spaced sites, and the endonucleolytic cleavage of genomic DNA; (DNA FRAGMENTATION); at internucleosomal sites. This mode of cell death serves as a balance to mitosis in regulating the size of animal tissues and in mediating pathologic processes associated with tumor growth.Information Services: Organized services to provide information on any questions an individual might have using databases and other sources. (From Random House Unabridged Dictionary, 2d ed)Consumer Health Information: Information intended for potential users of medical and healthcare services. There is an emphasis on self-care and preventive approaches as well as information for community-wide dissemination and use.G(M1) Ganglioside: A specific monosialoganglioside that accumulates abnormally within the nervous system due to a deficiency of GM1-b-galactosidase, resulting in GM1 gangliosidosis.Gangliosides: A subclass of ACIDIC GLYCOSPHINGOLIPIDS. They contain one or more sialic acid (N-ACETYLNEURAMINIC ACID) residues. Using the Svennerholm system of abbrevations, gangliosides are designated G for ganglioside, plus subscript M, D, or T for mono-, di-, or trisialo, respectively, the subscript letter being followed by a subscript arabic numeral to indicated sequence of migration in thin-layer chromatograms. (From Oxford Dictionary of Biochemistry and Molecular Biology, 1997)G(M3) Ganglioside: A ganglioside present in abnormally large amounts in the brain and liver due to a deficient biosynthetic enzyme, G(M3):UDP-N-acetylgalactosaminyltransferase. Deficiency of this enzyme prevents the formation of G(M2) ganglioside from G(M3) ganglioside and is the cause of an anabolic sphingolipidosis.Hyperbilirubinemia, Neonatal: Accumulation of BILIRUBIN, a breakdown product of HEME PROTEINS, in the BLOOD during the first weeks of life. This may lead to NEONATAL JAUNDICE. The excess bilirubin may exist in the unconjugated (indirect) or the conjugated (direct) form. The condition may be self-limiting (PHYSIOLOGICAL NEONATAL JAUNDICE) or pathological with toxic levels of bilirubin.Phototherapy: Treatment of disease by exposure to light, especially by variously concentrated light rays or specific wavelengths.Jaundice, Neonatal: Yellow discoloration of the SKIN; MUCOUS MEMBRANE; and SCLERA in the NEWBORN. It is a sign of NEONATAL HYPERBILIRUBINEMIA. Most cases are transient self-limiting (PHYSIOLOGICAL NEONATAL JAUNDICE) occurring in the first week of life, but some can be a sign of pathological disorders, particularly LIVER DISEASES.Bilirubin: A bile pigment that is a degradation product of HEME.Hyperbilirubinemia: A condition characterized by an abnormal increase of BILIRUBIN in the blood, which may result in JAUNDICE. Bilirubin, a breakdown product of HEME, is normally excreted in the BILE or further catabolized before excretion in the urine.Ursodeoxycholic Acid: An epimer of chenodeoxycholic acid. It is a mammalian bile acid found first in the bear and is apparently either a precursor or a product of chenodeoxycholate. Its administration changes the composition of bile and may dissolve gallstones. It is used as a cholagogue and choleretic.Kernicterus: A term used pathologically to describe BILIRUBIN staining of the BASAL GANGLIA; BRAIN STEM; and CEREBELLUM and clinically to describe a syndrome associated with HYPERBILIRUBINEMIA. Clinical features include athetosis, MUSCLE SPASTICITY or hypotonia, impaired vertical gaze, and DEAFNESS. Nonconjugated bilirubin enters the brain and acts as a neurotoxin, often in association with conditions that impair the BLOOD-BRAIN BARRIER (e.g., SEPSIS). This condition occurs primarily in neonates (INFANT, NEWBORN), but may rarely occur in adults. (Menkes, Textbook of Child Neurology, 5th ed, p613)
2000). "Human acid ceramidase is overexpressed but not mutated in prostate cancer". Genes Chromosomes Cancer. 29 (2): 137-46. ... 2001). "Human acid ceramidase: processing, glycosylation, and lysosomal targeting". J. Biol. Chem. 276 (38): 35352-60. doi: ... The ASAH1 gene encodes in humans the acid ceramidase enzyme. This gene encodes a heterodimeric protein consisting of a ... 2003). "Mutation analysis of the acid ceramidase gene in Japanese patients with Farber disease". J. Inherit. Metab. Dis. 25 (7 ...
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". ... A novel but highly conserved gene family of neutral/alkaline ceramidases". J. Biol. Chem. 275 (15): 11229-34. doi:10.1074/jbc. ...
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 ...
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 ...
However, carmofur is a highly potent acid ceramidase (AC) inhibitor. Ceramide influences cancer cell survival, growth and death ... "Discovery of highly potent acid ceramidase inhibitors with in vitro tumor chemosensitizing activity". Scientific Reports. 3 ( ...
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". ... "Ceramidases: regulators of cellular responses mediated by ceramide, sphingosine, and sphingosine-1-phosphate". Biochim. Biophys ...
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 ...
Normally, the enzyme ceramidase breaks down fatty material in the body's cells. In Farber Disease, the gene responsible for ... Farber disease (also known as Farber's lipogranulomatosis, ceramidase deficiency, "Fibrocytic dysmucopolysaccharidosis," and " ... autosomal recessive lysosomal storage disease marked by a deficiency in the enzyme ceramidase that causes an accumulation of ...
Sphingosine can be released from ceramides, a process catalyzed by the enzyme ceramidase. Phosphorylation of sphingosine is ...
... neutral ceramidase, and sphingolipid metabolites". Pediatr. Res. 61 (1): 61-6. doi:10.1203/01.pdr.0000250534.92934.c2. PMID ...
One of its treatments is a version of ceramidase being developed as treatment for Farber disease. Another was licensed from ...
NAAA's primary structure is nearly identical to that of acid ceramidase (AC), whose only difference is the substitution of Leu ... It does have 30% homology with acid ceramidase, sharing a significant portion of its amino acid sequence. NAAA was discovered ... 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 ...
... de formation via de novo sphinganine and fatty acyl-CoA and via sphingosine produced by the breakdown of ceramide by ceramidase ...
... genetic lysosomal disorder caused by a deficiency of the enzyme acid ceramidase. It has symptoms similar to JIA including ...
... 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 gamma interferon ionizing ...
... and they identified acid ceramidase as the enzyme deficient in Farber's disease. In 1964, Moser returned to the Joseph P. ...
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 ...
Ceramidase deficiency Ceramide trihexosidosis Ceraunophobia Cerebellar agenesis Cerebellar ataxia areflexia pes cavus optic ...
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. ...
... acid ceramidase) 1 ASPH: encoding enzyme Aspartyl/asparaginyl beta-hydroxylase AZIN1: encoding protein Antizyme inhibitor 1 ...
Compare neutral ceramidase ELISA Kits from leading suppliers on Biocompare. View specifications, prices, citations, reviews, ... Your search returned 9 neutral ceramidase ELISA ELISA Kit across 4 suppliers. ...
Acid Ceramidase Deficiency in Mice Results in a Broad Range of Central Nervous System Abnormalities. American Journal of ... Acid Ceramidase Deficiency in Mice Results in a Broad Range of Central Nervous System Abnormalities. / Sikora, Jakub; Dworski, ... Sikora, J., Dworski, S., Jones, E. E., Kamani, M. A., Micsenyi, M. C., Sawada, T., ... Walkley, S. U. (2017). Acid Ceramidase ... title = "Acid Ceramidase Deficiency in Mice Results in a Broad Range of Central Nervous System Abnormalities", ...
... and is structurally similar to acid ceramidase (1). NAAA is both a lysosomal and a secreted enzyme, and like acid ceramidase, ...
Farber disease (also known as Farbers lipogranulomatosis, ceramidase deficiency, "Fibrocytic dysmucopolysaccharidosis," and " ... Normally, the enzyme ceramidase breaks down fatty material in the bodys cells. In Farber Disease, the gene responsible for ... autosomal recessive lysosomal storage disease marked by a deficiency in the enzyme ceramidase that causes an accumulation of ...
... is an ultra-rare lysosomal storage disorder resulting from the inherited deficiency of the enzyme acid ceramidase, and the ...
Li CM, Park JH, He X, Levy B, Chen F, Arai K, Adler DA, Disteche CM, Koch J, Sandhoff K, et al (1999) The human acid ceramidase ... Eliyahu E, Shtraizent N, Shalgi R, Schuchman EH (2012) Construction of conditional ceramidase knockout mice and in vivo effects ... Acid ceramidase has also been shown to be involved in the resistance of certain tumor cells to chemotherapeutic agents and has ... Park JH, Schuchman EH (2006) Acid ceramidase and human disease. Biochim Biophys Acta 1758: 2133‐ 2138 ...
Presently, 7 human ceramidases encoded by 7 distinct genes have been cloned: acid ceramidase (ASAH1) - cell survival neutral ... Ceramidase at the US National Library of Medicine Medical Subject Headings (MeSH) EC 3.5.1.23 Molecular and Cellular Biology ... Ceramidase (EC 3.5.1.23, acylsphingosine deacylase, glycosphingolipid ceramide deacylase) is an enzyme which cleaves fatty ... UT Southwestern researchers showed that introducing ceramidase in diabetic mice returned their insulin sensitivity to normal. ...
The most potent in vitro inhibitor of acid ceramidase, B-13, recently revealed to be virtually inactive towards lysosomal acid ... Potent Inhibition of Acid Ceramidase by Novel B-13 Analogues. Denny Proksch, Jan Jasper Klein, and Christoph Arenz ... Indeed, inhibitors of acid ceramidase have been reported to induce cell death and to display potentiating effects to classical ... Herein, we report a novel B-13-derived scaffold for more effective inhibitors of acid ceramidase. Furthermore, we provide hints ...
Compare Acid Ceramidase ELISA Kits from leading suppliers on Biocompare. View specifications, prices, citations, reviews, and ... Acid Ceramidase ELISA Kits. The ELISA (enzyme-linked immunosorbent assay) is a widely used application for detecting and ... Your search returned 15 Acid Ceramidase ELISA ELISA Kit across 3 suppliers. ...
... Marie-Elaine ... "Blockade of Lysosomal Acid Ceramidase Induces GluN2B-Dependent Tau Phosphorylation in Rat Hippocampal Slices," Neural ...
Acid ceramidase subunit beta. B, D. 253. Homo sapiens. Mutation(s): 1 Gene Names: ASAH1, ASAH, HSD-33, HSD33. EC: 3.5.1.23 (PDB ... Acid ceramidase subunit alpha. A, C. 131. Homo sapiens. Mutation(s): 2 Gene Names: ASAH1, ASAH, HSD-33, HSD33. EC: 3.5.1.23 ( ... Human acid ceramidase (AC) is a lysosomal cysteine amidase, which has received a great deal of interest in recent years as a ... Human acid ceramidase (AC) is a lysosomal cysteine amidase, which has received a great deal of interest in recent years as a ...
IPR006823 Ceramidase_alk. IPR038445 NCDase_C_sf. IPR031331 NEUT/ALK_ceramidase_C. ... IPR006823 Ceramidase_alk. IPR038445 NCDase_C_sf. IPR031331 NEUT/ALK_ceramidase_C. ... Putative inactive neutral ceramidase BAdd BLAST. 165. Proteomic databases. PaxDb, a database of protein abundance averages ... Belongs to the neutral ceramidase family.Curated. Phylogenomic databases. evolutionary genealogy of genes: Non-supervised ...
Nonlysosomal ceramidase Ceramidase (CDase) is an enzyme that hydrolyzes the N-acyl linkage between a fatty acid and a sphingoid ... Cloning of a ceramidase homologue from Mycobacterium tuberculosis. J Biol Chem. 1999;274:36616-22.CrossRefPubMedGoogle Scholar ... A novel but highly conserved gene family of neutral/alkaline ceramidases. J Biol Chem. 2000b;275:11229-34.CrossRefPubMedGoogle ... Tani M., Ito M. (2018) Neutral Ceramidase. In: Choi S. (eds) Encyclopedia of Signaling Molecules. Springer, Cham. * .RIS Papers ...
Markedly perturbed hematopoiesis in acid ceramidase deficient mice. Shaalee Dworski, Alexandra Berger, Caren Furlonger, Joshua ... Markedly perturbed hematopoiesis in acid ceramidase deficient mice. Shaalee Dworski, Alexandra Berger, Caren Furlonger, Joshua ... Markedly perturbed hematopoiesis in acid ceramidase deficient mice Message Subject (Your Name) has forwarded a page to you from ...
Alkaline ceramidase YPC1Add BLAST. 316. Proteomic databases. PaxDb, a database of protein abundance averages across all three ... Alkaline ceramidase YPC1 (EC:3.5.1.-*Search proteins in UniProtKB for this EC number. ... "Characterization of yeast mutants lacking alkaline ceramidases YPC1 and YDC1.". Voynova N.S., Mallela S.K., Vazquez H.M., ... Belongs to the alkaline ceramidase family.Curated. Keywords - Domaini. Transmembrane, Transmembrane helix. Phylogenomic ...
... J Pathol. 2016 ... Here we reveal the essential role of alkaline ceramidase 1 (Acer1) in the skin. Acer1-deficient (Acer1(-/-) ) mice showed ... the levels of which are tightly regulated by a balance of ceramide synthase and ceramidase activities. ...
The ceramidase activity was in the linear range with time and protein concentration. Of note, the linear range with time was ... Because the observed ceramidase activity is low, amidase activity for other natural lipids cannot be ruled out at this stage. ... In this mechanism, which is similar to what was proposed for neutral ceramidase (ref. 12 in the main text), the zinc ion ... These data strongly suggest that all members of the PAQR family may have a ceramidase activity. (c) Sequence alignment of the ...
Biology the absence of a gene or a region of a chromosome normally present Explanation of Ceramidase deficiency ... Looking for Ceramidase deficiency? Find out information about Ceramidase deficiency. ... redirected from Ceramidase deficiency). Also found in: Dictionary, Thesaurus, Medical, Legal, Financial. deficiency. Biology ... Ceramidase deficiency , Article about Ceramidase deficiency by The Free Dictionary https://encyclopedia2.thefreedictionary.com/ ...
Here we report the identification of six novel mutations in the acid ceramidase gene causi … ... autosomal recessively inherited sphingolipid storage disorder due to the deficient activity of lysosomal acid ceramidase, ... Expression of the mutated acid ceramidase cDNAs in COS-1 cells and subsequent determination of acid ceramidase residual enzyme ... Molecular analysis of acid ceramidase deficiency in patients with Farber disease Hum Mutat. 2001 Mar;17(3):199-209. doi: ...
... Duan, Rui-Dong LU ; Verkade, H J; Cheng, ... Alkaline sphingomyelinase (Alk-SMase) and neutral ceramidase (N-CDase) in the intestinal microvillar membrane are responsible ... Alkaline sphingomyelinase (Alk-SMase) and neutral ceramidase (N-CDase) in the intestinal microvillar membrane are responsible ... article{2fea1906-449d-4cca-ac43-8bace27c1bba, abstract = {Alkaline sphingomyelinase (Alk-SMase) and neutral ceramidase (N-CDase ...
Abstract 5484: Targeted inhibition of acid ceramidase and C6-ceramide exposure induces synergistic killing in cancer cells. ... Abstract 5484: Targeted inhibition of acid ceramidase and C6-ceramide exposure induces synergistic killing in cancer cells ... Abstract 5484: Targeted inhibition of acid ceramidase and C6-ceramide exposure induces synergistic killing in cancer cells ... Abstract 5484: Targeted inhibition of acid ceramidase and C6-ceramide exposure induces synergistic killing in cancer cells ...
In ceramide metabolism, ceramidases catalyze the breakdown of ceramide to sphingosine and the inhibition of ceramidase activity ... H9 cells, a human T cell lymphoma cell line, were cultured in medium with the acid ceramidase inhibitor N-oleoyl-ethanolamine ( ... Herein, we report that modulation of ceramide metabolism by extended treatment with a ceramidase inhibitor allows cells to ... extended treatment with an acid ceramidase inhibitor modulates the metabolism of ceramide and selects for a radiation-resistant ...
... ... These cells accumulate ceramide as the result of an inherited deficiency of acidic ceramidase. We demonstrate that Farber ... absence, activation, Apoptosis, caspase-3, cd95, ceramidase, Ceramide, death, Farber disease, fas, gd3 ganglioside, Mice, ...
Mouse Alkaline ceramidase 1 (ACER1) ELISA Kit-NP_597999.1 (MBS7235867) product datasheet at MyBioSource, ELISA Kits ... ACER1 elisa kit :: Mouse Alkaline ceramidase 1 (ACER1) ELISA Kit. Catalog #. MBS7235867 .mycenter { display: block; margin-left ... alkaline ceramidase 1; alkCDase 1; alkaline CDase 1; acylsphingosine deacylase 3; N-acylsphingosine amidohydrolase 3; N- ... Belongs to the alkaline ceramidase family.. Protein type: Hydrolase; Lipid Metabolism - sphingolipid; EC 3.5.1.23; Endoplasmic ...
The synthesis of a novel fluorogenic ceramidase substrate, and its processing both in vitro and in cultured cells in a ... The combinatorial chemistry approach for the discovery of acid ceramidase inhibitors requires the availability of a high- ... Similarly, hydrolysis occurred in intact cultured cells that overexpressed acidic ceramidase. The assay was validated for the ... identification and characterization of acidic ceramidase inhibitors by using several alpha-ketoamide ceramide analogues, whose ...
  • Here, we describe a micelleplex system based on an amphiphilic and cationic triblock copolymer, which can systemically deliver siRNA targeting the acid ceramidase (AC) gene for cancer therapy. (naver.com)
  • Furthermore, we provide hints for an introduction of basic functional groups at an alternative site of the B-13 scaffold that do not interfere with acid ceramidase inhibition in vitro . (hindawi.com)
  • Metabolic labeling studies in fibroblasts of four patients showed that even though acid ceramidase precursor protein was synthesized in these individuals, rapid proteolysis of the mutated, mature acid ceramidase occurred within the lysosome. (nih.gov)
  • Here, we show that expression of ceramidase facilitates the dissolution of incompletely formed and inappropriately located elements of rhabdomeric membranes in ninaE(I17) mutants lacking the G protein receptor Rh1 in R1-R6 photoreceptor cells. (umassmed.edu)
  • E2 binds to the protein acid ceramidase, which sets off a chain of cellular processes that suppress angiogenesis. (yonsei.ac.kr)
  • Adiponectin receptors (ADIPORs) are integral membrane proteins controlling glucose and lipid metabolism by mediating, at least in part, a cellular ceramidase activity 1 which catalyzes the hydrolysis of ceramide to produce sphingosine and a free fatty acid (FFA). (pubmedcentralcanada.ca)
  • These results suggest that the absence of intracellular accumulation of ceramide following irradiation, through the modulation of ceramide metabolism using an acid ceramidase inhibitor for extended periods of time in culture, represents one potential mechanism for the development of radioresistance. (aacrjournals.org)
  • Ceramide, a key molecule in the metabolism of sphingolipids, is converted by ceramidase to sphingosine, and phosphorylated by ceramide kinase to form ceramide-1-phosphate (C1P). (chat-shuffle.net)
  • These represent the first inducible genetic models that acutely manipulate adiponectin receptor signaling in adult mouse tissues, which allows us to directly assess AdipoR signaling on glucose and lipid metabolism.Overexpression of either adiponectin receptor isoform in the adipocyte or hepatocyte is sufficient to enhance ceramidase activity, whole body glucose metabolism, and hepatic insulin sensitivity, while opposing hepatic steatosis. (hepaton.com)
  • When challenged with a leptin-deficient genetic model of type 2 diabetes, AdipoR2 expression in adipose or liver is sufficient to reverse hyperglycemia and glucose intolerance.These observations reveal that adiponectin is critical for AdipoR-induced ceramidase activation which enhances hepatic glucose and lipid metabolism via rapidly acting 'cross-talk' between liver and adipose tissue sphingolipids. (hepaton.com)
  • Indeed, inhibitors of acid ceramidase have been reported to induce cell death and to display potentiating effects to classical radio- or chemo therapy in a number of in vitro and in vivo cancer models. (hindawi.com)
  • H9 cells, a human T cell lymphoma cell line, were cultured in medium with the acid ceramidase inhibitor N-oleoyl-ethanolamine (NOE), starting at a concentration level of 10 μM, with gradual increases to an NOE concentration of 100 μM. (aacrjournals.org)
  • Cloning of a ceramidase homologue from Mycobacterium tuberculosis. (springer.com)