AnatomyOrganismsDiseasesChemicals and DrugsAnalytical, Diagnostic and Therapeutic Techniques and EquipmentPhenomena and ProcessesTechnology, Industry, AgricultureInformation Science
Serine C-PalmitoyltransferaseHereditary Sensory and Autonomic NeuropathiesGlycine HydroxymethyltransferaseCarnitine O-PalmitoyltransferaseMalonyl Coenzyme APalmitoyl Coenzyme AAcyltransferasesSerineAcyl Coenzyme ACarnitineSphingolipidsCarnitine AcyltransferasesMitochondria, LiverEpoxy CompoundsPalmitoylcarnitinePalmitatesStarvationFatty AcidsSerine EndopeptidasesPalmitic AcidKetone BodiesLiverCeramidesSubmitochondrial ParticlesMyoglobinuriaRats, Inbred StrainsLipoylationOxidation-ReductionMolecular Sequence DataSphingomonasAmino Acid SequenceKineticsSerine ProteasesSphingosine N-AcyltransferaseSphingosineSerine Proteinase InhibitorsCarnitine O-AcetyltransferaseMitochondria, MuscleLipid Metabolism, Inborn ErrorsIsoenzymesCoenzyme APalmitic AcidsDigitoninMicrobodiesCitrate (si)-SynthaseCycloserineIntracellular MembranesSphingomyelinsFatty Acids, MonounsaturatedMitochondria, HeartCaprylatesSubstrate SpecificityLipid MetabolismAcetylcarnitineAzetidinesGene Expression Regulation, EnzymologicEthers, CyclicAcetyl-CoA CarboxylaseRats, Inbred BUFBase SequencePhosphorylationOleic AcidsMitochondriaOleic AcidCeramidasesFumonisinsSequence Homology, Amino AcidAcyl-CoA OxidaseRNA, MessengerPichia3-Hydroxyacyl CoA DehydrogenasesMutationMuscle, SkeletalGlyoxylatesBinding SitesEsterificationBenzyl AlcoholAcyl-CoA Dehydrogenase, Long-ChainCoenzyme A LigasesDetergentsRats, WistarCatalysisMyocardiumBezafibrateCells, CulturedSphingomyelin PhosphodiesteraseBlotting, WesternTriglyceridesDiabetes Mellitus, ExperimentalCell LineClofibrateEnzyme InhibitorsRhabdomyolysisDiabetic KetoacidosisMutagenesis, Site-DirectedDNA, ComplementaryRats, Sprague-DawleyMicrosomesPPAR alphaAcyl-CoA Dehydrogenase
Subunit1.50BiosynthesisCarnitineApolipoprotein B mRNA editinSynthesis of sphingolipidsRate-limitiInhibitionSphingolipidsSphingolipid MetabolismCeramidePhosphateNovoProteinsCatalyzesPathwayActivityMetabolicBasesSmallHumanSequenceFunctionBiosynthesisTransferaseSubunit of serine palmitoyltransferaseCondensationSynthesisMetabolic DiseasesMetabolismSubunitsProteinCarnitineThreonineAmide-linkedSPTLC2InhibitorChainImmunoassayEthanolamineSPTLC1LipidSpeciesSubstrateHyperprolinemiaCharacterizationReactionMoleculesSandwichAcidComplexFoundAffect
Subunit9
  • The SPTLC1 gene provides instructions for making one part (subunit) of an enzyme called serine palmitoyltransferase (SPT). (medlineplus.gov)
  • SPTLC1 gene mutations reduce the amount of functional SPTLC1 subunit that is produced, which results in an SPT enzyme with altered activity. (medlineplus.gov)
  • Dawkins JL, Hulme DJ, Brahmbhatt SB, Auer-Grumbach M, Nicholson GA. Mutations in SPTLC1, encoding serine palmitoyltransferase, long chain base subunit-1, cause hereditary sensory neuropathy type I. Nat Genet. (medlineplus.gov)
  • Elevation of 20-carbon long chain bases due to a mutation in serine palmitoyltransferase small subunit b results in neurodegeneration. (jax.org)
  • Description: This is Double-antibody Sandwich Enzyme-linked immunosorbent assay for detection of Human Serine Palmitoyltransferase, Long Chain Base Subunit 3 (SPTLC3) in tissue homogenates, cell lysates and other biological fluids. (1elisakits.com)
  • Description: Enzyme-linked immunosorbent assay based on the Double-antibody Sandwich method for detection of Human Serine Palmitoyltransferase, Long Chain Base Subunit 3 (SPTLC3) in samples from tissue homogenates, cell lysates and other biological fluids with no significant corss-reactivity with analogues from other species. (1elisakits.com)
  • Description: A sandwich ELISA kit for detection of Serine Palmitoyltransferase, Long Chain Base Subunit 3 from Human in samples from blood, serum, plasma, cell culture fluid and other biological fluids. (1elisakits.com)
  • Description: A sandwich quantitative ELISA assay kit for detection of Mouse Serine Palmitoyltransferase, Long Chain Base Subunit 3 (SPTLC3) in samples from tissue homogenates, cell lysates or other biological fluids. (1elisakits.com)
  • There are also 2 highly related isoforms of a third subunit, SSSPTA (MIM 613540) and SSSPTB (MIM 610412), that confer acyl-CoA preference of the SPT enzyme and are essential for maximal enzyme activity (Han et al. (utsouthwestern.edu)
1.502
  • A small family of fungal enzymes, built based on the validated sequence from Gibberella (Fusarium verticilloides) and closely resembling Serine C-palmitoyltransferases (family 2.3.1.50b). (unipr.it)
  • Serine C-palmitoyltransferase (EC 2.3.1.50 ) from yeast (genes LCB1 and LCB2), which catalyzes the condensation of palmitoyl-CoA and serine to form 3-ketosphinganine. (expasy.org)
Biosynthesis1
  • 8-amino-7-oxononanoate synthase (EC 2.3.1.47 ) (7-KAP synthase), a bacterial enzyme (gene bioF) which catalyzes an intermediate step in the biosynthesis of biotin: the addition of 6-carboxy-hexanoyl-CoA to alanine to form 8- amino-7-oxononanoate. (expasy.org)
Carnitine1
  • Longer-term (24 and 48 h) treatment reduced the expression of lipogenic markers (FA synthase and sterol regulatory element-binding protein-1) and increased the expression of lipolytic [hormone-sensitive lipase (HSL) and adipose triglyceride lipase (ATGL)] and mitochondrial (peroxisome proliferator-activated receptor γ coactivator-1α and carnitine palmitoyltransferase 1) markers. (academic-accelerator.com)
Apolipoprotein B mRNA editin1
Synthesis of sphingolipids2
  • [ 3 ] SPT is the rate-limiting enzyme in synthesis of sphingolipids, including ceramide and sphingomyelin. (medscape.com)
  • Myriocin, a fungal metabolite isolated from Myriococcum albomyces, Isaria sinclairi, and Mycelia sterilia, is a potent inhibitor of serine palmitoyltransferase (SPT), a key enzyme in de novo synthesis of sphingolipids. (cdc.gov)
Rate-limiti1
  • Following myocardial infarction (MI), serine palmitoyl transferase (SPT), the rate-limiting enzyme of the de novo pathway of ceramide synthesis, and ceramides were found increased. (jci.org)
Inhibition1
  • Inhibition of serine palmitoyltransferase by myriocin, a natural mycotoxin, causes induction of c-myc in mouse liver. (cdc.gov)
Sphingolipids4
  • The SPT enzyme is involved in making certain fats called sphingolipids. (medlineplus.gov)
  • Although the SPT enzyme does not produce a normal amount of sphingolipids, the body is able to compensate, and there does not seem to be an overall reduction of these fats in the body. (medlineplus.gov)
  • The precursors of sphingolipids, called long-chain bases, from which all sphingolipids are derived, are of a certain length, 18 carbons, but they can also be found at 16- and 20-carbon lengths, with the length being controlled by a multi-protein enzyme called serine palmitoyltransferase (SPT). (jax.org)
  • For simpleness, degradative enzymes (blue) for reutilization of sphingolipids in the salvage pathway are included but these reactions happen in various subcellular compartments (find Fig. 2). (moonphase2018.com)
Sphingolipid Metabolism2
  • Initial, a lot of the regulatory enzymes and proteins involved with sphingolipid metabolism as well as the receptors for S1P have already been cloned. (moonphase2018.com)
  • Lipid metabolism.sphingolipid metabolism.serine. (ntu.edu.sg)
Ceramide1
  • That is accompanied by some reactions (catalysed by enzymes in crimson) resulting in development of ceramide and following development of sphingomyelin and glycosphingolipids. (moonphase2018.com)
Phosphate3
  • A parallel Kennedy pathway forms phosphatidylethanolamine from ethanolamine - the only difference being a different enzyme, ethanolamine-phosphate cytidylyltransferase, catalyzing the second step. (smpdb.ca)
  • Aminotransferases share certain mechanistic features with other pyridoxal-phosphate dependent enzymes, such as the covalent binding of the pyridoxal-phosphate group to a lysine residue. (expasy.org)
  • The sequence around the pyridoxal-phosphate attachment site of this class of enzyme is sufficiently conserved to allow the creation of a specific pattern. (expasy.org)
Novo1
  • De novo sphingolipid synthesis begins with the formation of 3-keto-dihydrosphingosine by serine palmitoyltransferase (SPT). (frontiersin.org)
Proteins1
Catalyzes1
  • β-galactosidase , also called lactase , beta-gal or β-gal , is a glycoside hydrolase enzyme that catalyzes the hydrolysis of β-galactosides into monosaccharides through the breaking of a glycosidic bond . (wikidoc.org)
Pathway3
  • The first reaction of the Kennedy pathway involves the cytosol-localized enzyme choline/ethanolamine kinase catalyzing the conversion of choline into phosphocholine. (smpdb.ca)
  • The sphingolipid biosynthetic pathway begins in the endoplasmic reticulum, where membrane enzyme complexes catalyze the first step of the process. (stjude.org)
  • By contrast, the hepatic expression of sorbitol dehydrogenase, the second enzyme for the polyol pathway, is quite high [ 8 ]. (hindawi.com)
Activity1
  • Recent work in his lab led to the discovery of a new genetic form of ALS that stems from excessive activity of an enzyme called serine palmitoyltransferase. (livelikelou.org)
Metabolic1
  • A complex metabolic network consisting of enzymes catalyzing their synthesis, modification (phosphorylation, glycosylation) and breakdown regulates accumulation of sphingolipid species and thereby the sphingolipid pool at rheostat conditions, and this can undergo substantial changes in response to metabolic and external challenges. (encyclopedia.pub)
Bases1
  • This altered enzyme makes molecules called deoxysphingoid bases, which it does not normally produce. (medlineplus.gov)
Small1
Human2
  • It is an essential enzyme in the human body. (wikidoc.org)
  • Beta-galactose is used in such dairy products as yogurt, sour cream, and some cheeses which are treated with the enzyme to break down any lactose before human consumption. (wikidoc.org)
Sequence1
  • On the basis of sequence similarity, these various enzymes can be grouped [ 1 ] into subfamilies. (expasy.org)
Function1
  • Many adult humans lack the lactase enzyme, which has the same function of beta-gal, so they are not able to properly digest dairy products. (wikidoc.org)
Biosynthesis7
  • This gene encodes a subunit of the serine palmitoyltransferase complex which catalyzes the rate-limiting step in sphingolipid biosynthesis. (nih.gov)
  • 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. (nih.gov)
  • EC 2.3.1.50) catalyzes the condensation of serine with palmitoyl-CoA to form 3-ketosphinganine in the first step of de novo sphingolipid biosynthesis. (unipr.it)
  • We show for the frst time that Chlamydomonas expresses serine palmitoyl transferase (SPT), the frst enzyme in (phyto)ceramide biosynthesis. (uky.edu)
  • use DICARBOXYLIC ACIDS 1970-1979 MH - 3-Phosphoshikimate 1-Carboxyvinyltransferase UI - D051229 MN - D8.811.913.225.735 MS - An enzyme of the shikimate pathway of AROMATIC AMINO ACID biosynthesis, it generates 5-enolpyruvylshikimate 3-phosphate and ORTHOPHOSPHATE from PHOSPHOENOLPYRUVATE and SHIKIMATE-3-PHOSPHATE. (nih.gov)
  • The enzyme is a key regulatory enzyme in heme biosynthesis. (wakehealth.edu)
  • Our goal is to identify the metabolic pathways between lipid synthesis and antioxidant defense involved in FAHFA biosynthesis, to examine the substrate specifity of the candidate enzyme, and to introduce a methodology for the quantification of metabolic fluxes through redox reactions linking central carbon metabolism and nicotinamidadene cofactor-driven reductive biosynthesis. (cas.cz)
Transferase3
  • The associations between individual plasma SFAs, serine palmitoyl-transferase long-chain base subunit 3 gene rs680379 polymorphism, and type 2 diabetes among Chinese adults. (nih.gov)
  • 2012). There is also evidence that AICD regulates sphingolipid synthesis via serine-palmitoyl transferase (Grimm et al. (bibf1120.com)
  • An enzyme of the transferase class that catalyzes condensation of the succinyl group from succinyl coenzyme A with glycine to form delta-aminolevulinate. (wakehealth.edu)
Subunit of serine palmitoyltransferase1
  • An overexpression screen of Nicotiana benthamiana cDNAs identified a gene for the LCB2 subunit of serine palmitoyltransferase (SPT) as a potent inducer of hypersensitive response-like cell death. (apsnet.org)
Condensation2
  • This enzyme KOS953 ic50 catalyzes the limiting first step in the formation of ceramides: the condensation of palmitoyl-CoA and serine, making 3-ketosphinganine. (bioinbrief.com)
  • Condensation of serine and palmitoyl-CoA (or myristoyl-CoA) which forms 3-ketosphinganine is certainly then reduced to create dihydrosphingosine by NADH-dependent reductase. (healthy-nutrition-plan.com)
Synthesis8
  • [ 3 ] SPT is the rate-limiting enzyme in synthesis of sphingolipids, including ceramide and sphingomyelin. (medscape.com)
  • When bred to mice that express tissue-specific Cre recombinase, resulting offspring will overexpression the first enzyme of the sphingolipid metabolism pathway resulting in elevation of sphingolipid synthesis. (nih.gov)
  • ceramide synthesis pathway and to dissect in which hypothalamic nucleus those actions take place, we targeted serine palmitoyltransferase long chain foundation subunit 1 (SPTLC1). (bioinbrief.com)
  • Sphingolipid amounts are tightly governed by these enzymes, permitting them to work as bioactive mediators.De novosphingolipid synthesis starts using the enzyme serine palmitoyltransferase (SPT). (healthy-nutrition-plan.com)
  • Increased production of ceramides is linked to elevated activity of serine palmitoyltransferase, the de novo ceramide synthesis pathway enzyme, in the presence of high glucose levels [6], while decreased activity of acid ceramidase, an enzyme responsible for degradation of ceramide, has been also reported in the hyperglycemia conditions in association with increased inflammatory response [7]. (scientificarchives.com)
  • De novo sphingolipid synthesis begins with formation of 3-keto-dihydrosphingosine by serine palmitoyltransferase . (cloudfront.net)
  • An important enzyme in the glyoxylic acid cycle which reversibly catalyzes the synthesis of L-malate from acetyl-CoA and glyoxylate. (nih.gov)
  • Enzymes that catalyze the synthesis of FATTY ACIDS from acetyl-CoA and malonyl-CoA derivatives. (lookformedical.com)
Metabolic Diseases1
  • Serine Palmitoyltransferase Subunit 3 and Metabolic Diseases. (nih.gov)
Metabolism1
  • AICD also BI6727 enzyme inhibitor has a direct link to lipid metabolism as it has been found to suppress the expression of the major lipoprotein receptor LRP1 and as such affect apoE/cholesterol metabolism BI6727 enzyme inhibitor (Liu et al. (bibf1120.com)
Subunits1
  • The enzyme consists of two different subunits. (nih.gov)
Protein1
  • Within the cell, the SPT enzyme is mainly found on the endoplasmic reticulum, which is a structure involved in protein processing and transport. (medlineplus.gov)
Carnitine1
  • Amongst these, carnitine palmitoyltransferase 1C (CPT1C) has been demonstrated to form an integral part of native AMPAR complexes in brain tissue extracts. (frontiersin.org)
Threonine1
Amide-linked1
  • In this compound (named 'flavolipin'), serine is amide-linked to 3-hydroxyisoheptadecanoic acid which is esterified to isopentadecanoic acid. (gerli.com)
SPTLC21
  • The mice carry a transgene that is a single-chain version of serine palmitoyltransferase with its three core (human) subunit cDNAs, SPTLC2 , SPTSSA , and SPTLC1 fused into a single reading frame linked T2A-separated EGFP under control of a promoter that is activated by Cre recombinase expression (Stop-fSPT). (nih.gov)
Inhibitor1
  • These research have demonstrated useful and structural variety BI6727 enzyme inhibitor of lipid rafts and characterized in the plasma membrane of Jurkat T cells the SM-rich domains which acquired spatial and useful. (bibf1120.com)
Chain5
  • Dawkins JL, Hulme DJ, Brahmbhatt SB, Auer-Grumbach M, Nicholson GA. Mutations in SPTLC1, encoding serine palmitoyltransferase, long chain base subunit-1, cause hereditary sensory neuropathy type I. Nat Genet. (medlineplus.gov)
  • Description: This is Double-antibody Sandwich Enzyme-linked immunosorbent assay for detection of Mouse Serine Palmitoyltransferase, Long Chain Base Subunit 3 (SPTLC3) in Tissue homogenates, cell lysates and other biological fluids. (operatiebrp.nl)
  • Description: A sandwich ELISA kit for detection of Serine Palmitoyltransferase, Long Chain Base Subunit 3 from Mouse in samples from blood, serum, plasma, cell culture fluid and other biological fluids. (operatiebrp.nl)
  • Description: A sandwich quantitative ELISA assay kit for detection of Human Serine Palmitoyltransferase, Long Chain Base Subunit 3 (SPTLC3) in samples from tissue homogenates or other biological fluids. (operatiebrp.nl)
  • [11] The six CerS enzymes have different specificity for acyl-CoA substrates, resulting in the generation of dihydroceramides with differing chain lengths (ranging from C14-C26). (cloudfront.net)
Immunoassay2
  • GH ELISA kit applies the quantitative sandwich enzyme immunoassay technique. (elisakit.cc)
  • A commutability confirmation test for the blood aldosterone measurement was performed on liquid chromatography-mass spectrometry/mass spectrometry (LC-MS/MS) as a designated comparison method (DCM) and four chemiluminescent enzyme immunoassay (CLEIA) measurement procedures based on metrological traceability. (stemcellcharter.org)
Ethanolamine1
SPTLC12
  • The SPTLC1 gene provides instructions for making one part (subunit) of an enzyme called serine palmitoyltransferase (SPT). (medlineplus.gov)
  • SPTLC1 gene mutations reduce the amount of functional SPTLC1 subunit that is produced, which results in an SPT enzyme with altered activity. (medlineplus.gov)
Lipid1
  • A divergent Pseudomonas aeruginosa palmitoyltransferase essential for cystic fibrosis-specific lipid A . 91:158-174. (mcmaster.ca)
Species1
  • However, studies have demonstrated that serine palmitoyltransferase has some activity toward other species of fatty acyl-CoA [8] and alternative amino acids , [9] and the diversity of sphingoid bases has recently been reviewed. (cloudfront.net)
Substrate2
  • The enzyme (HRP) and substrate are allowed to react over a short incubation period. (elisakit.cc)
  • The enzyme-substrate reaction is terminated by addition of a sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450 nm. (elisakit.cc)
Hyperprolinemia1
  • Defects in the enzyme are the cause of hyperprolinemia II. (nih.gov)
Characterization2
  • Cloning and initial characterization of a new subunit for mammalian serine-palmitoyltransferase. (nih.gov)
  • Hornemann T, Richard S, Rutti MF, Wei Y, von Eckardstein A. Cloning and initial characterization of a new subunit for mammalian serine-palmitoyltransferase. (medlineplus.gov)
Reaction1
Molecules2
  • The SPT enzyme initiates the first step of sphingolipid production, in which the molecules serine and palmitoyl CoA combine to form a molecule called ketodihydrosphingosine. (medlineplus.gov)
  • This altered enzyme makes molecules called deoxysphingoid bases, which it does not normally produce. (medlineplus.gov)
Sandwich1
  • Description: This is Double-antibody Sandwich Enzyme-linked immunosorbent assay for detection of Human Phosphodiesterase 10A (PDE10A) in Tissue homogenates and other biological fluids. (lotusbiotechnologies.com)
Acid1
Complex1
  • Covalent attachment via an isopeptide bond to its substrates requires prior activation by the E1 complex SAE1-SAE2 and linkage to the E2 enzyme UBE2I, and can be promoted by an E3 ligase such as PIAS1-4, RANBP2 or CBX4. (nih.gov)
Found1
  • The structure of Geobacillus stearothermophilus purine nucleoside phosphorylase, an enzyme of biocatalytic interest, is reported and was found to include the presence of an N-terminal tag in the active site of each subunit that belongs to the other subunit in each dimer. (nih.gov)
Affect1
  • Nevertheless, despite its potential as a depalmitoylating enzyme, CPT1C does not affect the palmitoylation state of GluA1. (frontiersin.org)