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Protein

Cystathionine beta-synthase-like protein

Gene

CBSL

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

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. Also involved in the production of hydrogen sulfide, a gasotransmitter with signaling and cytoprotective effects on neurons.By similarity

Caution

There is a duplication of the CBS gene on chromosome 21. CBS, which was originally identified as the gene encoding cystathionine beta-synthase (AC P35520), and CBSL encode identical proteins.Curated

Catalytic activityi

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

Cofactori

pyridoxal 5'-phosphateBy similarity

Activity regulationi

Allosterically activated by S-adenosyl-methionine/AdoMet. Activated by S-adenosylhomocysteine/AdoHcy. Binds non-covalently to a heme group that may control the redox sensitivity of the enzyme.By similarity

Pathwayi: L-cysteine biosynthesis

This protein is involved in step 1 of the subpathway that synthesizes L-cysteine from L-homocysteine and L-serine.By similarity
Proteins known to be involved in the 2 steps of the subpathway in this organism are:
  1. Cystathionine beta-synthase (CBS), Cystathionine beta-synthase-like protein (CBSL)
  2. Cystathionine gamma-lyase (CTH)
This subpathway is part of the pathway L-cysteine biosynthesis, which is itself part of Amino-acid biosynthesis.
View all proteins of this organism that are known to be involved in the subpathway that synthesizes L-cysteine from L-homocysteine and L-serine, the pathway L-cysteine biosynthesis and in Amino-acid biosynthesis.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi52Iron (heme axial ligand)By similarity1
Metal bindingi65Iron (heme axial ligand)By similarity1
Binding sitei149Pyridoxal phosphateBy similarity1
Binding sitei349Pyridoxal phosphateBy similarity1

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionLyase
Biological processAmino-acid biosynthesis, Cysteine biosynthesis
LigandHeme, Iron, Metal-binding, Pyridoxal phosphate

Enzyme and pathway databases

UniPathwayi
UPA00136;UER00201

Names & Taxonomyi

Protein namesi
Recommended name:
Cystathionine beta-synthase-like proteinCurated (EC:4.2.1.22By similarity)
Alternative name(s):
Beta-thionaseBy similarity
Serine sulfhydraseBy similarity
Gene namesi
Name:CBSLImported
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 21

Organism-specific databases

HGNCiHGNC:51829 CBSL
neXtProtiNX_P0DN79

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Cytoplasm, Nucleus

Pathology & Biotechi

Organism-specific databases

DisGeNETi102724560
875
OpenTargetsiENSG00000160200
ENSG00000274276

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00004353951 – 551Cystathionine beta-synthase-like proteinAdd BLAST551

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei27PhosphoserineCombined sources1
Modified residuei119N6-(pyridoxal phosphate)lysineBy similarity1
Modified residuei199PhosphoserineCombined sources1
Cross-linki211Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO)By similarity

Keywords - PTMi

Isopeptide bond, Phosphoprotein, Ubl conjugation

Proteomic databases

EPDiP0DN79
PaxDbiP0DN79
PeptideAtlasiP0DN79
PRIDEiP0DN79

PTM databases

iPTMnetiP0DN79
PhosphoSitePlusiP0DN79

Expressioni

Organism-specific databases

HPAiHPA001223

Interactioni

Subunit structurei

Homotetramer.By similarity

Protein-protein interaction databases

IntActiP0DN79, 2 interactors
STRINGi9606.ENSP00000344460

Chemistry databases

BindingDBiP0DN79

Structurei

3D structure databases

ProteinModelPortaliP0DN79
SMRiP0DN79
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini418 – 476CBSPROSITE-ProRule annotationAdd BLAST59

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni256 – 260Pyridoxal phosphate bindingBy similarity5

Sequence similaritiesi

Keywords - Domaini

CBS domain

Phylogenomic databases

KOiK01697
OMAiMTLVDWT
PhylomeDBiP0DN79

Family and domain databases

InterProiView protein in InterPro
IPR000644 CBS_dom
IPR005857 Cysta_beta_synth
IPR001216 P-phosphate_BS
IPR001926 PLP-dep
IPR036052 Trypto_synt_PLP_dependent
PfamiView protein in Pfam
PF00571 CBS, 1 hit
PF00291 PALP, 1 hit
SMARTiView protein in SMART
SM00116 CBS, 1 hit
SUPFAMiSSF53686 SSF53686, 1 hit
TIGRFAMsiTIGR01137 cysta_beta, 1 hit
PROSITEiView protein in PROSITE
PS51371 CBS, 1 hit
PS00901 CYS_SYNTHASE, 1 hit

Sequencei

Sequence statusi: Complete.

P0DN79-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MPSETPQAEV GPTGCPHRSG PHSAKGSLEK GSPEDKEAKE PLWIRPDAPS
60 70 80 90 100
RCTWQLGRPA SESPHHHTAP AKSPKILPDI LKKIGDTPMV RINKIGKKFG
110 120 130 140 150
LKCELLAKCE FFNAGGSVKD RISLRMIEDA ERDGTLKPGD TIIEPTSGNT
160 170 180 190 200
GIGLALAAAV RGYRCIIVMP EKMSSEKVDV LRALGAEIVR TPTNARFDSP
210 220 230 240 250
ESHVGVAWRL KNEIPNSHIL DQYRNASNPL AHYDTTADEI LQQCDGKLDM
260 270 280 290 300
LVASVGTGGT ITGIARKLKE KCPGCRIIGV DPEGSILAEP EELNQTEQTT
310 320 330 340 350
YEVEGIGYDF IPTVLDRTVV DKWFKSNDEE AFTFARMLIA QEGLLCGGSA
360 370 380 390 400
GSTVAVAVKA AQELQEGQRC VVILPDSVRN YMTKFLSDRW MLQKGFLKEE
410 420 430 440 450
DLTEKKPWWW HLRVQELGLS APLTVLPTIT CGHTIEILRE KGFDQAPVVD
460 470 480 490 500
EAGVILGMVT LGNMLSSLLA GKVQPSDQVG KVIYKQFKQI RLTDTLGRLS
510 520 530 540 550
HILEMDHFAL VVHEQIQYHS TGKSSQRQMV FGVVTAIDLL NFVAAQERDQ

K
Length:551
Mass (Da):60,587
Last modified:February 17, 2016 - v1
Checksum:iF89E69C67BDE6701
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
FP236240 Genomic DNA No translation available.
CCDSiCCDS82646.1
RefSeqiNP_000062.1, NM_000071.2
NP_001171479.1, NM_001178008.2
NP_001171480.1, NM_001178009.2
NP_001307227.1, NM_001320298.1
NP_001308002.1, NM_001321073.1
XP_011544401.1, XM_011546099.1
XP_016883703.1, XM_017028214.1
XP_016883704.1, XM_017028215.1
XP_016883705.1, XM_017028216.1
XP_016883706.1, XM_017028217.1
XP_016883707.1, XM_017028218.1
XP_016883979.1, XM_017028490.1
XP_016883980.1, XM_017028491.1
XP_016883981.1, XM_017028492.1
UniGeneiHs.533013

Genome annotation databases

EnsembliENST00000398168; ENSP00000381234; ENSG00000274276
ENST00000617706; ENSP00000481719; ENSG00000274276
ENST00000618024; ENSP00000479559; ENSG00000274276
ENST00000624406; ENSP00000485669; ENSG00000274276
GeneIDi102724560
875
KEGGihsa:102724560
hsa:875

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
FP236240 Genomic DNA No translation available.
CCDSiCCDS82646.1
RefSeqiNP_000062.1, NM_000071.2
NP_001171479.1, NM_001178008.2
NP_001171480.1, NM_001178009.2
NP_001307227.1, NM_001320298.1
NP_001308002.1, NM_001321073.1
XP_011544401.1, XM_011546099.1
XP_016883703.1, XM_017028214.1
XP_016883704.1, XM_017028215.1
XP_016883705.1, XM_017028216.1
XP_016883706.1, XM_017028217.1
XP_016883707.1, XM_017028218.1
XP_016883979.1, XM_017028490.1
XP_016883980.1, XM_017028491.1
XP_016883981.1, XM_017028492.1
UniGeneiHs.533013

3D structure databases

ProteinModelPortaliP0DN79
SMRiP0DN79
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

IntActiP0DN79, 2 interactors
STRINGi9606.ENSP00000344460

Chemistry databases

BindingDBiP0DN79

PTM databases

iPTMnetiP0DN79
PhosphoSitePlusiP0DN79

Proteomic databases

EPDiP0DN79
PaxDbiP0DN79
PeptideAtlasiP0DN79
PRIDEiP0DN79

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000398168; ENSP00000381234; ENSG00000274276
ENST00000617706; ENSP00000481719; ENSG00000274276
ENST00000618024; ENSP00000479559; ENSG00000274276
ENST00000624406; ENSP00000485669; ENSG00000274276
GeneIDi102724560
875
KEGGihsa:102724560
hsa:875

Organism-specific databases

CTDi102724560
875
DisGeNETi102724560
875
GeneCardsiCBSL
HGNCiHGNC:51829 CBSL
HPAiHPA001223
neXtProtiNX_P0DN79
OpenTargetsiENSG00000160200
ENSG00000274276
GenAtlasiSearch...

Phylogenomic databases

KOiK01697
OMAiMTLVDWT
PhylomeDBiP0DN79

Enzyme and pathway databases

UniPathwayi
UPA00136;UER00201

Miscellaneous databases

PROiPR:P0DN79

Family and domain databases

InterProiView protein in InterPro
IPR000644 CBS_dom
IPR005857 Cysta_beta_synth
IPR001216 P-phosphate_BS
IPR001926 PLP-dep
IPR036052 Trypto_synt_PLP_dependent
PfamiView protein in Pfam
PF00571 CBS, 1 hit
PF00291 PALP, 1 hit
SMARTiView protein in SMART
SM00116 CBS, 1 hit
SUPFAMiSSF53686 SSF53686, 1 hit
TIGRFAMsiTIGR01137 cysta_beta, 1 hit
PROSITEiView protein in PROSITE
PS51371 CBS, 1 hit
PS00901 CYS_SYNTHASE, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiCBSL_HUMAN
AccessioniPrimary (citable) accession number: P0DN79
Entry historyiIntegrated into UniProtKB/Swiss-Prot: February 17, 2016
Last sequence update: February 17, 2016
Last modified: November 7, 2018
This is version 20 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  3. Human chromosome 21
    Human chromosome 21: entries, gene names and cross-references to MIM
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Main funding by: National Institutes of Health

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