Protein: Cystathionine beta-synthase (EC 4.2.1.22) (Beta-thionase) (Serine sulfhydrase) (Q91WT9)
Organism: Mus musculus (Mouse)
Function: Hydro-lyase catalyzing the first step of the transsulfuration pathway, where the hydroxyl group of L-serine is displaced by L-homocysteine in a beta-replacement reaction to form L-cystathionine, the precursor of L-cysteine. This catabolic route allows the elimination of L-methionine and the toxic metabolite L-homocysteine (By similarity). Also involved in the production of hydrogen sulfide, a gasotransmitter with signaling and cytoprotective effects on neurons (By similarity).
Subcellular location: Cytoplasm {ECO:0000250|UniProtKB:P35520}. Nucleus {ECO:0000250|UniProtKB:P35520}.
Sequence: MPSGTSQCEDGSAGGFQHLDMHSEKRQLEKGPSGDKDRVWIRPDTPSRCTWQLGRAMADSPHYHTVLTKSPKILPDILRKIGNTPMVRINKISKNAGLKCELLAKCEFFNAGGSVKDRISLRMIEDAERAGNLKPGDTIIEPTSGNTGIGLALAAAVKGYRCIIVMPEKMSMEKVDVLRALGAEIVRTPTNARFDSPESHVGVAWRLKNEIPNSHILDQYRNASNPLAHYDDTAEEILQQCDGKLDMLVASAGTGGTITGIARKLKEKCPGCKIIGVDPEGSILAEPEELNQTEQTAYEVEGIGYDFIPTVLDRAVVDKWFKSNDEDSFAFARMLIAQEGLLCGGSSGSAMAVAVKAARELQEGQRCVVILPDSVRNYMSKFLSDKWMLQKGFMKEELSVKRPWWWRLRVQELSLSAPLTVLPTVTCEDTIAILREKGFDQAPVVNESGAILGMVTLGNMLSSLLAGKVRPSDEVCKVLYKQFKPIHLTDTLGTLSHILEMDHFALVVHEQIQSRDQAWSGVVGGPTDCSNGMSSKQQMVFGVVTAIDLLNFVAAREQTQT
InterPro domains: - IPR005857: Cystathionine beta-synthase (family) [74-554]
- IPR001216: Cysteine synthase/cystathionine beta-synthase, pyridoxal-phosphate attachment site (binding_site) [105-123]
- IPR036052: Tryptophan synthase beta chain-like, PALP domain superfamily (homologous_superfamily) [46-392]
- IPR001926: Tryptophan synthase beta chain-like, PALP domain (domain) [78-373]
- IPR046342: CBS domain superfamily (homologous_superfamily) [394-561]
- IPR046353: Cystathionine beta-synthase, C-terminal domain (domain) [408-552]
- IPR000644: CBS domain (domain) [412-474]
- IPR050214: Cysteine synthase/Cystathionine beta-synthase (family) [75-390]
Interaction partners: - Cystathionine gamma-lyase
- Betaine--homocysteine S-methyltransferase 1
- Methionine synthase
- Adenosylhomocysteinase
- Serine hydroxymethyltransferase, cytosolic
- Serine hydroxymethyltransferase, mitochondrial
- S-methylmethionine--homocysteine S-methyltransferase BHMT2
- Phosphoserine phosphatase
- Probable cysteine--tRNA ligase, mitochondrial
- Putative adenosylhomocysteinase 3
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0003824 (catalytic activity), GO:0016829 (lyase activity), GO:0016835 (carbon-oxygen lyase activity), GO:0016836 (hydro-lyase activity)
Biological Process (BP): GO:0008150 (biological process), GO:0008152 (metabolic process), GO:0065007 (biological regulation), GO:0050896 (response to stimulus), GO:0050789 (regulation of biological process), GO:0009987 (cellular process), GO:0048519 (negative regulation of biological process), GO:0009058 (biosynthetic process), GO:0044237 (cellular metabolic process), GO:0044281 (small molecule metabolic process), GO:0006807 (nitrogen compound metabolic process), GO:0050794 (regulation of cellular process), GO:0006950 (response to stress), GO:0071704 (organic substance metabolic process), GO:0048523 (negative regulation of cellular process), GO:0044238 (primary metabolic process), GO:1901564 (organonitrogen compound metabolic process), GO:1901576 (organic substance biosynthetic process), GO:0044283 (small molecule biosynthetic process), GO:0060548 (negative regulation of cell death), GO:0010941 (regulation of cell death), GO:0006082 (organic acid metabolic process), GO:0044249 (cellular biosynthetic process), GO:0006520 (amino acid metabolic process), GO:0006790 (sulfur compound metabolic process), GO:1901566 (organonitrogen compound biosynthetic process), GO:0000096 (sulfur amino acid metabolic process), GO:0044272 (sulfur compound biosynthetic process), GO:0008652 (amino acid biosynthetic process), GO:0016053 (organic acid biosynthetic process), GO:0043436 (oxoacid metabolic process), GO:1901605 (alpha-amino acid metabolic process), GO:0019752 (carboxylic acid metabolic process), GO:1901607 (alpha-amino acid biosynthetic process), GO:0050667 (homocysteine metabolic process), GO:0006534 (cysteine metabolic process), GO:0000097 (sulfur amino acid biosynthetic process), GO:0009069 (serine family amino acid metabolic process), GO:0046394 (carboxylic acid biosynthetic process), GO:0009070 (serine family amino acid biosynthetic process), GO:0009092 (homoserine metabolic process), GO:0019344 (cysteine biosynthetic process), GO:0006563 (L-serine metabolic process), GO:0006535 (cysteine biosynthetic process from serine)
Cellular Component (CC): GO:0005575 (cellular component), GO:0110165 (cellular anatomical entity), GO:0005829 (cytosol), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0005737 (cytoplasm), GO:0043229 (intracellular organelle), GO:0043227 (membrane-bounded organelle), GO:0043231 (intracellular membrane-bounded organelle), GO:0005634 (nucleus)