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CystathionineCystathionine beta-SynthaseCystathionine gamma-LyaseAdenylosuccinate LyaseArgininosuccinic AciduriaSteroid 17-alpha-HydroxylaseAdrenal Hyperplasia, CongenitalOxo-Acid-LyasesMeglutolHomocystinuriaArgininosuccinate LyasePurine-Pyrimidine Metabolism, Inborn ErrorsAldehyde-Lyases46, XY Disorders of Sex DevelopmentAmino Acid Metabolism, Inborn ErrorsHydrogen SulfideConsanguinityMetabolism, Inborn ErrorsCytochromes b5Carbon-Oxygen LyasesLyasesATP Citrate (pro-S)-LyaseHomocysteineHomozygoteMethionineMutation, MissenseHyperhomocysteinemiaS-AdenosylmethionineCysteineCysteine SynthasePyridoxal PhosphateVitamin B 6 DeficiencySulfurPolysaccharide-LyasesPedigreeHomocystineHydro-LyasesAlkynesBetaine-Homocysteine S-MethyltransferasePyridoxineMolecular Sequence DataSulfidesVitamin B 12 DeficiencyHomoserineFolic Acid DeficiencyS-AdenosylhomocysteineSerine O-AcetyltransferaseMutationVitamin A DeficiencyAmino Acid SequenceDeficiency DiseasesBase SequenceMice, Knockoutalpha 1-Antitrypsin DeficiencyLiver5-Methyltetrahydrofolate-Homocysteine S-MethyltransferaseSulfur IsotopesKineticsVitamin UCloning, MolecularBetaineVitamin B 12Reflex, AbdominalAmino Acids, SulfurEscherichia coliGamma RaysFolic AcidMice, Inbred C57BLSerineVitamin B 6Vitamin D DeficiencyGlycineHemeIgA DeficiencyRecombinant ProteinsHeterozygoteThiamine DeficiencyL-Serine DehydrataseRNA, MessengerAmino AcidsGene Expression Regulation, EnzymologicMethylmalonic AcidGlucosephosphate Dehydrogenase DeficiencyLactobacillus helveticusCells, CulturedCystineSulfhydryl CompoundsMagnesium DeficiencyCatalysisModels, MolecularAnemia, Iron-DeficiencyVitamin E DeficiencyDisease Models, AnimalSequence Homology, Amino AcidAscorbic Acid DeficiencyImmunologic Deficiency SyndromesVisceral PainPhenotypeDietCatalytic Domain

SynthaseEnzymeCysteineEnzymesTranssulfurationProteinHumanActivityBeta-synthaseCystathionaseCDNAMitochondrialAmmoniaHomoserineCatalyzesGeneMutationsAminoDisorderSulfurElectronEffects

Synthase7

• Cystathionine gamma lyase also shows gamma-synthase activity depending on the concentrations of reactants present. (wikipedia.org)
• In the gamma synthase mechanism, the gamma carbon is attacked by a sulfur nucleophile, resulting in the formation of a new sulfur-gamma carbon bond. (wikipedia.org)
• Other members include cystathionine γ synthase, cystathionine β lyase, and methionine γ lyase. (wikipedia.org)
• Vascular complications of cystathionine beta-synthase deficiency: future directions for homocysteine-to-hydrogen sulfide research. (esmed.org)
• Activation of cystathionine synthase by adenosylmethionine and adenosylethionine. (esmed.org)
• Reversible heme-dependent regulation of human cystathionine beta-synthase by a flavoprotein oxidoreductase. (esmed.org)
• Elevated endogenous estrogens stimulate human uterine artery endothelial cell (hUAEC) hydrogen sulfide (H 2 S) production by selectively upregulating the expression of H 2 S synthesizing enzyme cystathionine β-synthase (CBS), but the underlying mechanisms are underdetermined. (bioscientifica.com)

Enzyme4

• 2-oxobutanoate-forming)) breaks down cystathionine into cysteine, 2-oxobutanoate (α-ketobutyrate), and ammonia: L-cystathionine + H2O = L-cysteine + 2-oxobutanoate + NH3 (overall reaction) (1a) L-cystathionine = L-cysteine + 2-aminobut-2-enoate (1b) 2-aminobut-2-enoate = 2-iminobutanoate (spontaneous) (1c) 2-iminobutanoate + H2O = 2-oxobutanoate + NH3 (spontaneous) Pyridoxal phosphate is a prosthetic group of this enzyme. (wikipedia.org)
• Cystathionine γ-lyase also catalyses the following elimination reactions: L-homoserine to form H2O, NH3 and 2-oxobutanoate L-cystine, producing thiocysteine, pyruvate and NH3 L-cysteine producing pyruvate, NH3 and H2S In some bacteria and mammals, including humans, this enzyme takes part in generating hydrogen sulfide. (wikipedia.org)
• Mechanistically, p53 acetylation and translocation were, at least partially, attributed to the loss of the vasoprotective enzyme, CSE (cystathionine γ-lyase). (bvsalud.org)
• The results showed that the endogenous H2S-producing enzyme cystathione-γ-lyase (CSE) protein expression was downregulated in renal artery tissue, plasma H2S levels were reduced. (bvsalud.org)

Cysteine5

• Cystathionase uses pyridoxal phosphate to facilitate the cleavage of the sulfur-gamma carbon bond of cystathionine, resulting in the release of cysteine. (wikipedia.org)
• To reform the aldimine, this lone pair pushes back down, cleaving the sulfur-gamma carbon bond, resulting in the release of cysteine. (wikipedia.org)
• cystathionine gamma-lyase , cysteine dioxygenase , and cysteine sulfinic acid decarboxylase. (cloudfront.net)
• Under such metabolic stress conditions, supplies of L-cysteine for glutathione synthesis become limiting, so homocysteine is diverted from the transmethylation pathway (which forms methionine) into the transsulfuration pathway (which forms cystathionine). (pathbank.org)
• 2-Hydroxybutyrate is released as a byproduct when cystathionine is cleaved into cysteine that is incorporated into glutathione. (pathbank.org)

Enzymes2

• Cystathionine γ-lyase is a member of the Cys/Met metabolism PLP-dependent enzymes family. (wikipedia.org)
• Like many other PLP-dependent enzymes, cystathionine γ-lyase is a tetramer with D2 symmetry. (wikipedia.org)

Transsulfuration1

• Taurine is also produced by the transsulfuration pathway , which converts homocysteine into cystathionine . (cloudfront.net)

Protein1

• This gastrointestinal dysfunction can, in turn, perpetuate histidine deficiency and therefore impair all protein digestion. (healthmatters.io)

Human1

• Estradiol-17β (E 2 β) stimulated CBS but not cystathionine γ-lyase (CSE) expression in pregnant human uterine artery ex vivo , which was attenuated by the estrogen receptor (ER) antagonist ICI 182,780. (bioscientifica.com)

Activity2

• Deficiencies in cystathionase activity have also been shown to contribute to glutathione depletion in patients with cancer and AIDS. (wikipedia.org)
• Factors controlling the expressed activity of histidine ammonia-lyase in the epidermis and the resulting accumulation of urocanic acid. (healthmatters.io)

Beta-synthase1

• Cystathionine beta synthase catalyzes the upper reaction and cystathionine gamma-lyase catalyzes the lower reaction. (iiab.me)

Cystathionase6

• Cystathionase uses pyridoxal phosphate to facilitate the cleavage of the sulfur-gamma carbon bond of cystathionine, resulting in the release of cysteine. (wikipedia.org)
• Deficiencies in cystathionase activity have also been shown to contribute to glutathione depletion in patients with cancer and AIDS. (wikipedia.org)
• Mutations and deficiencies in cystathionase are associated with cystathioninuria. (wikipedia.org)
• Gamma-cystathionase deficiency, also called cystathionase deficiency, is an autosomal recessive metabolic disorder caused by a defective cystathionine gamma-lyase. (pathbank.org)
• Since there is currently no cure forGamma-cystathionase deficiency, treatment involves managing the disorder's symptoms. (pathbank.org)
• Pascal TA, Gaull GE, Beratis NG, Gillam BM, Tallan HH: Cystathionase deficiency: evidence for genetic heterogeneity in primary cystathioninuria. (pathbank.org)

CDNA1

• Lu Y, O'Dowd BF, Orrego H, Israel Y: Cloning and nucleotide sequence of human liver cDNA encoding for cystathionine gamma-lyase. (smpdb.ca)

Mitochondrial1

• IGF2 deficiency causes mitochondrial defects in skeletal muscle. (molcells.org)

Ammonia2

• 2-oxobutanoate-forming)) breaks down cystathionine into cysteine, 2-oxobutanoate (α-ketobutyrate), and ammonia: L-cystathionine + H2O = L-cysteine + 2-oxobutanoate + NH3 (overall reaction) (1a) L-cystathionine = L-cysteine + 2-aminobut-2-enoate (1b) 2-aminobut-2-enoate = 2-iminobutanoate (spontaneous) (1c) 2-iminobutanoate + H2O = 2-oxobutanoate + NH3 (spontaneous) Pyridoxal phosphate is a prosthetic group of this enzyme. (wikipedia.org)
• In the final step in the biosynthesis of cysteine it catalyzes the cleavage of cystathionine to yield cysteine, ammonia, and 2-ketobutyrate. (bvsalud.org)

Homoserine1

• Cystathionine γ-lyase also catalyses the following elimination reactions: L-homoserine to form H2O, NH3 and 2-oxobutanoate L-cystine, producing thiocysteine, pyruvate and NH3 L-cysteine producing pyruvate, NH3 and H2S In some bacteria and mammals, including humans, this enzyme takes part in generating hydrogen sulfide. (wikipedia.org)

Catalyzes1

• Cystathionine gamma-lyase catalyzes the conversion of L-Serine and homocysteine into L-Cystathionine which is the substrate of cystathionine gamma-lyase. (pathbank.org)

Gene1

• Relationship between cystathionine gamma-lyase gene polymorphism and essential hypertension in Northern Chinese Han population. (cdc.gov)

Mutations1

• Wang J, Hegele RA: Genomic basis of cystathioninuria (MIM 219500) revealed by multiple mutations in cystathionine gamma-lyase (CTH). (smpdb.ca)

Amino1

• The amino group on cystathionine is deprotonated and undergoes a nucleophilic attack of the internal aldimine. (wikipedia.org)

Disorder2

• This disorder is characterized by a large accumulation of L-Cystathionine in the cell. (pathbank.org)
• Symptoms of the disorder include mental deficiency and seizure. (pathbank.org)

Sulfur2

• To reform the aldimine, this lone pair pushes back down, cleaving the sulfur-gamma carbon bond, resulting in the release of cysteine. (wikipedia.org)
• Our findings reveal an unexpected negative interaction between MR, sulfur deficiency and antitumour immunity and further uncover a vital role of gut microbiota in mediating this interaction. (bvsalud.org)

Electron1

• The lone pair from the pyridine nitrogen pushes electron density to the gamma carbon, which is protonated by lysine. (wikipedia.org)

Effects1

• Effects in Cancer Cells of the Recombinant l-Methionine Gamma-Lyase from Brevibacterium aurantiacum. (aspetjournals.org)