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Protein

Cystathionine beta-synthase

Gene

Cbs

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at transcript leveli

Functioni

Only known pyridoxal phosphate-dependent enzyme that contains heme. Important regulator of hydrogen sulfide, especially in the brain, utilizing cysteine instead of serine to catalyze the formation of hydrogen sulfide. Hydrogen sulfide is a gastratransmitter with signaling and cytoprotective effects such as acting as a neuromodulator in the brain to protect neurons against hypoxic injury (By similarity).By similarity

Catalytic activityi

L-serine + L-homocysteine = L-cystathionine + H2O.

Cofactori

Pathwayi

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Metal bindingi49 – 491Iron (heme axial ligand)By similarity
Metal bindingi62 – 621Iron (heme axial ligand)By similarity
Binding sitei146 – 1461Pyridoxal phosphateBy similarity
Binding sitei346 – 3461Pyridoxal phosphateBy similarity

GO - Molecular functioni

  1. cystathionine beta-synthase activity Source: MGI
  2. enzyme binding Source: MGI
  3. heme binding Source: MGI
  4. identical protein binding Source: MGI
  5. metal ion binding Source: UniProtKB-KW
  6. modified amino acid binding Source: MGI
  7. protein homodimerization activity Source: MGI
  8. pyridoxal phosphate binding Source: MGI
  9. ubiquitin protein ligase binding Source: MGI

GO - Biological processi

  1. blood vessel remodeling Source: MGI
  2. cartilage development involved in endochondral bone morphogenesis Source: MGI
  3. cellular response to hypoxia Source: Ensembl
  4. cerebellum morphogenesis Source: MGI
  5. cysteine biosynthetic process Source: MGI
  6. cysteine biosynthetic process from serine Source: InterPro
  7. cysteine biosynthetic process via cystathionine Source: GO_Central
  8. endochondral ossification Source: MGI
  9. homocysteine metabolic process Source: MGI
  10. hydrogen sulfide biosynthetic process Source: UniProtKB
  11. maternal process involved in female pregnancy Source: MGI
  12. negative regulation of apoptotic process Source: Ensembl
  13. regulation of blood vessel size Source: MGI
  14. regulation of cGMP metabolic process Source: MGI
  15. regulation of JUN kinase activity Source: MGI
  16. response to folic acid Source: MGI
  17. superoxide metabolic process Source: MGI
  18. transsulfuration Source: Ensembl
Complete GO annotation...

Keywords - Molecular functioni

Lyase

Keywords - Biological processi

Amino-acid biosynthesis, Cysteine biosynthesis

Keywords - Ligandi

Heme, Iron, Metal-binding, Pyridoxal phosphate

Enzyme and pathway databases

BRENDAi4.2.1.22. 3474.
ReactomeiREACT_273450. Cysteine formation from homocysteine.
UniPathwayiUPA00136; UER00201.

Names & Taxonomyi

Protein namesi
Recommended name:
Cystathionine beta-synthase (EC:4.2.1.22)
Alternative name(s):
Beta-thionase
Serine sulfhydrase
Gene namesi
Name:Cbs
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
ProteomesiUP000000589 Componenti: Chromosome 17

Organism-specific databases

MGIiMGI:88285. Cbs.

Subcellular locationi

  1. Nucleus By similarity
  2. Cytoplasm By similarity

GO - Cellular componenti

  1. cytoplasm Source: GO_Central
  2. cytosol Source: MGI
  3. intracellular membrane-bounded organelle Source: MGI
  4. nucleolus Source: MGI
  5. nucleus Source: MGI
Complete GO annotation...

Keywords - Cellular componenti

Cytoplasm, Nucleus

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Initiator methioninei1 – 11RemovedBy similarity
Chaini2 – 561560Cystathionine beta-synthasePRO_0000262592Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei116 – 1161N6-(pyridoxal phosphate)lysineBy similarity
Cross-linki208 – 208Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO)By similarity

Post-translational modificationi

Binds covalently to a heme group through a thiolate ligand.By similarity

Keywords - PTMi

Isopeptide bond, Ubl conjugation

Proteomic databases

MaxQBiQ91WT9.
PaxDbiQ91WT9.
PRIDEiQ91WT9.

PTM databases

PhosphoSiteiQ91WT9.

Expressioni

Gene expression databases

BgeeiQ91WT9.
CleanExiMM_CBS.
ExpressionAtlasiQ91WT9. baseline and differential.
GenevestigatoriQ91WT9.

Interactioni

Subunit structurei

Homotetramer.By similarity

Protein-protein interaction databases

DIPiDIP-60823N.
IntActiQ91WT9. 1 interaction.
MINTiMINT-4121506.
STRINGi10090.ENSMUSP00000066878.

Structurei

3D structure databases

SMRiQ91WT9. Positions 40-558.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini414 – 47461CBSPROSITE-ProRule annotationAdd
BLAST

Region

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Regioni253 – 2575Pyridoxal phosphate bindingBy similarity

Sequence similaritiesi

Contains 1 CBS domain.PROSITE-ProRule annotation

Keywords - Domaini

CBS domain

Phylogenomic databases

eggNOGiCOG0031.
GeneTreeiENSGT00510000047027.
HOGENOMiHOG000217392.
HOVERGENiHBG000918.
InParanoidiQ91WT9.
KOiK01697.
OMAiWMASYGF.
OrthoDBiEOG7J70F1.
PhylomeDBiQ91WT9.
TreeFamiTF300784.

Family and domain databases

InterProiIPR000644. CBS_dom.
IPR005857. Cysta_beta_synth.
IPR001216. P-phosphate_BS.
IPR001926. TrpB-like_PLP-dep.
[Graphical view]
PfamiPF00571. CBS. 1 hit.
PF00291. PALP. 1 hit.
[Graphical view]
SMARTiSM00116. CBS. 1 hit.
[Graphical view]
SUPFAMiSSF53686. SSF53686. 1 hit.
TIGRFAMsiTIGR01137. cysta_beta. 1 hit.
PROSITEiPS51371. CBS. 1 hit.
PS00901. CYS_SYNTHASE. 1 hit.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q91WT9-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MPSGTSQCED GSAGGFQHLD MHSEKRQLEK GPSGDKDRVW IRPDTPSRCT
60 70 80 90 100
WQLGRAMADS PHYHTVLTKS PKILPDILRK IGNTPMVRIN KISKNAGLKC
110 120 130 140 150
ELLAKCEFFN AGGSVKDRIS LRMIEDAERA GNLKPGDTII EPTSGNTGIG
160 170 180 190 200
LALAAAVKGY RCIIVMPEKM SMEKVDVLRA LGAEIVRTPT NARFDSPESH
210 220 230 240 250
VGVAWRLKNE IPNSHILDQY RNASNPLAHY DDTAEEILQQ CDGKLDMLVA
260 270 280 290 300
SAGTGGTITG IARKLKEKCP GCKIIGVDPE GSILAEPEEL NQTEQTAYEV
310 320 330 340 350
EGIGYDFIPT VLDRAVVDKW FKSNDEDSFA FARMLIAQEG LLCGGSSGSA
360 370 380 390 400
MAVAVKAARE LQEGQRCVVI LPDSVRNYMS KFLSDKWMLQ KGFMKEELSV
410 420 430 440 450
KRPWWWRLRV QELSLSAPLT VLPTVTCEDT IAILREKGFD QAPVVNESGA
460 470 480 490 500
ILGMVTLGNM LSSLLAGKVR PSDEVCKVLY KQFKPIHLTD TLGTLSHILE
510 520 530 540 550
MDHFALVVHE QIQSRDQAWS GVVGGPTDCS NGMSSKQQMV FGVVTAIDLL
560
NFVAAREQTQ T
Length:561
Mass (Da):61,544
Last modified:January 23, 2007 - v3
Checksum:i072CF81AFE10372E
GO
Isoform 2 (identifier: Q91WT9-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     514-528: SRDQAWSGVVGGPTD → Y

Show »
Length:547
Mass (Da):60,193
Checksum:i5DC0F6E3DAED8461
GO

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei514 – 52815SRDQA…GGPTD → Y in isoform 2. 2 PublicationsVSP_021790Add
BLAST

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK077669 mRNA. Translation: BAC36943.1.
BC013472 mRNA. Translation: AAH13472.1.
BC013480 mRNA. Translation: AAH13480.1.
BC026595 mRNA. Translation: AAH26595.1.
CCDSiCCDS28609.1. [Q91WT9-1]
CCDS28610.1. [Q91WT9-2]
RefSeqiNP_001258282.1. NM_001271353.1. [Q91WT9-2]
NP_659104.1. NM_144855.3. [Q91WT9-1]
NP_835742.1. NM_178224.3. [Q91WT9-2]
XP_006523612.1. XM_006523549.2. [Q91WT9-1]
XP_006523613.1. XM_006523550.2. [Q91WT9-1]
XP_006523614.1. XM_006523551.2. [Q91WT9-1]
UniGeneiMm.206417.
Mm.486781.

Genome annotation databases

EnsembliENSMUST00000067801; ENSMUSP00000066878; ENSMUSG00000024039. [Q91WT9-1]
ENSMUST00000078509; ENSMUSP00000077597; ENSMUSG00000024039. [Q91WT9-2]
ENSMUST00000118504; ENSMUSP00000113209; ENSMUSG00000024039. [Q91WT9-2]
GeneIDi12411.
KEGGimmu:12411.
UCSCiuc008bvl.1. mouse. [Q91WT9-1]
uc008bvm.1. mouse. [Q91WT9-2]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK077669 mRNA. Translation: BAC36943.1.
BC013472 mRNA. Translation: AAH13472.1.
BC013480 mRNA. Translation: AAH13480.1.
BC026595 mRNA. Translation: AAH26595.1.
CCDSiCCDS28609.1. [Q91WT9-1]
CCDS28610.1. [Q91WT9-2]
RefSeqiNP_001258282.1. NM_001271353.1. [Q91WT9-2]
NP_659104.1. NM_144855.3. [Q91WT9-1]
NP_835742.1. NM_178224.3. [Q91WT9-2]
XP_006523612.1. XM_006523549.2. [Q91WT9-1]
XP_006523613.1. XM_006523550.2. [Q91WT9-1]
XP_006523614.1. XM_006523551.2. [Q91WT9-1]
UniGeneiMm.206417.
Mm.486781.

3D structure databases

SMRiQ91WT9. Positions 40-558.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

DIPiDIP-60823N.
IntActiQ91WT9. 1 interaction.
MINTiMINT-4121506.
STRINGi10090.ENSMUSP00000066878.

PTM databases

PhosphoSiteiQ91WT9.

Proteomic databases

MaxQBiQ91WT9.
PaxDbiQ91WT9.
PRIDEiQ91WT9.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000067801; ENSMUSP00000066878; ENSMUSG00000024039. [Q91WT9-1]
ENSMUST00000078509; ENSMUSP00000077597; ENSMUSG00000024039. [Q91WT9-2]
ENSMUST00000118504; ENSMUSP00000113209; ENSMUSG00000024039. [Q91WT9-2]
GeneIDi12411.
KEGGimmu:12411.
UCSCiuc008bvl.1. mouse. [Q91WT9-1]
uc008bvm.1. mouse. [Q91WT9-2]

Organism-specific databases

CTDi875.
MGIiMGI:88285. Cbs.

Phylogenomic databases

eggNOGiCOG0031.
GeneTreeiENSGT00510000047027.
HOGENOMiHOG000217392.
HOVERGENiHBG000918.
InParanoidiQ91WT9.
KOiK01697.
OMAiWMASYGF.
OrthoDBiEOG7J70F1.
PhylomeDBiQ91WT9.
TreeFamiTF300784.

Enzyme and pathway databases

UniPathwayiUPA00136; UER00201.
BRENDAi4.2.1.22. 3474.
ReactomeiREACT_273450. Cysteine formation from homocysteine.

Miscellaneous databases

ChiTaRSiCbs. mouse.
NextBioi281194.
PROiQ91WT9.
SOURCEiSearch...

Gene expression databases

BgeeiQ91WT9.
CleanExiMM_CBS.
ExpressionAtlasiQ91WT9. baseline and differential.
GenevestigatoriQ91WT9.

Family and domain databases

InterProiIPR000644. CBS_dom.
IPR005857. Cysta_beta_synth.
IPR001216. P-phosphate_BS.
IPR001926. TrpB-like_PLP-dep.
[Graphical view]
PfamiPF00571. CBS. 1 hit.
PF00291. PALP. 1 hit.
[Graphical view]
SMARTiSM00116. CBS. 1 hit.
[Graphical view]
SUPFAMiSSF53686. SSF53686. 1 hit.
TIGRFAMsiTIGR01137. cysta_beta. 1 hit.
PROSITEiPS51371. CBS. 1 hit.
PS00901. CYS_SYNTHASE. 1 hit.
[Graphical view]
ProtoNetiSearch...

Publicationsi

  1. "The transcriptional landscape of the mammalian genome."
    Carninci P., Kasukawa T., Katayama S., Gough J., Frith M.C., Maeda N., Oyama R., Ravasi T., Lenhard B., Wells C., Kodzius R., Shimokawa K., Bajic V.B., Brenner S.E., Batalov S., Forrest A.R., Zavolan M., Davis M.J.
    , Wilming L.G., Aidinis V., Allen J.E., Ambesi-Impiombato A., Apweiler R., Aturaliya R.N., Bailey T.L., Bansal M., Baxter L., Beisel K.W., Bersano T., Bono H., Chalk A.M., Chiu K.P., Choudhary V., Christoffels A., Clutterbuck D.R., Crowe M.L., Dalla E., Dalrymple B.P., de Bono B., Della Gatta G., di Bernardo D., Down T., Engstrom P., Fagiolini M., Faulkner G., Fletcher C.F., Fukushima T., Furuno M., Futaki S., Gariboldi M., Georgii-Hemming P., Gingeras T.R., Gojobori T., Green R.E., Gustincich S., Harbers M., Hayashi Y., Hensch T.K., Hirokawa N., Hill D., Huminiecki L., Iacono M., Ikeo K., Iwama A., Ishikawa T., Jakt M., Kanapin A., Katoh M., Kawasawa Y., Kelso J., Kitamura H., Kitano H., Kollias G., Krishnan S.P., Kruger A., Kummerfeld S.K., Kurochkin I.V., Lareau L.F., Lazarevic D., Lipovich L., Liu J., Liuni S., McWilliam S., Madan Babu M., Madera M., Marchionni L., Matsuda H., Matsuzawa S., Miki H., Mignone F., Miyake S., Morris K., Mottagui-Tabar S., Mulder N., Nakano N., Nakauchi H., Ng P., Nilsson R., Nishiguchi S., Nishikawa S., Nori F., Ohara O., Okazaki Y., Orlando V., Pang K.C., Pavan W.J., Pavesi G., Pesole G., Petrovsky N., Piazza S., Reed J., Reid J.F., Ring B.Z., Ringwald M., Rost B., Ruan Y., Salzberg S.L., Sandelin A., Schneider C., Schoenbach C., Sekiguchi K., Semple C.A., Seno S., Sessa L., Sheng Y., Shibata Y., Shimada H., Shimada K., Silva D., Sinclair B., Sperling S., Stupka E., Sugiura K., Sultana R., Takenaka Y., Taki K., Tammoja K., Tan S.L., Tang S., Taylor M.S., Tegner J., Teichmann S.A., Ueda H.R., van Nimwegen E., Verardo R., Wei C.L., Yagi K., Yamanishi H., Zabarovsky E., Zhu S., Zimmer A., Hide W., Bult C., Grimmond S.M., Teasdale R.D., Liu E.T., Brusic V., Quackenbush J., Wahlestedt C., Mattick J.S., Hume D.A., Kai C., Sasaki D., Tomaru Y., Fukuda S., Kanamori-Katayama M., Suzuki M., Aoki J., Arakawa T., Iida J., Imamura K., Itoh M., Kato T., Kawaji H., Kawagashira N., Kawashima T., Kojima M., Kondo S., Konno H., Nakano K., Ninomiya N., Nishio T., Okada M., Plessy C., Shibata K., Shiraki T., Suzuki S., Tagami M., Waki K., Watahiki A., Okamura-Oho Y., Suzuki H., Kawai J., Hayashizaki Y.
    Science 309:1559-1563(2005) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 2).
    Strain: C57BL/6J.
  2. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORMS 1 AND 2).
    Tissue: Kidney.

Entry informationi

Entry nameiCBS_MOUSE
AccessioniPrimary (citable) accession number: Q91WT9
Secondary accession number(s): Q91WU3
Entry historyi
Integrated into UniProtKB/Swiss-Prot: November 28, 2006
Last sequence update: January 23, 2007
Last modified: April 1, 2015
This is version 111 of the entry and version 3 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  3. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into Uniref entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.