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Protein

Cystathionine gamma-lyase

Gene

CTH

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the ‘protein existence’ evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

Catalyzes the last step in the trans-sulfuration pathway from methionine to cysteine. Has broad substrate specificity. Converts cystathionine to cysteine, ammonia and 2-oxobutanoate. Converts two cysteine molecules to lanthionine and hydrogen sulfide. Can also accept homocysteine as substrate. Specificity depends on the levels of the endogenous substrates. Generates the endogenous signaling molecule hydrogen sulfide (H2S), and so contributes to the regulation of blood pressure. Acts as a cysteine-protein sulfhydrase by mediating sulfhydration of target proteins: sulfhydration consists of converting -SH groups into -SSH on specific cysteine residues of target proteins such as GAPDH, PTPN1 and NF-kappa-B subunit RELA, thereby regulating their function.3 Publications

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">Function</a> section describes the catalytic activity of an enzyme, i.e. a chemical reaction that the enzyme catalyzes.<p><a href='/help/catalytic_activity' target='_top'>More...</a></p>Catalytic activityi

<p>This subsection of the ‘Function’ section provides information relevant to cofactors. A cofactor is any non-protein substance required for a protein to be catalytically active. Some cofactors are inorganic, such as the metal atoms zinc, iron, and copper in various oxidation states. Others, such as most vitamins, are organic.<p><a href='/help/cofactor' target='_top'>More...</a></p>Cofactori

pyridoxal 5'-phosphate3 Publications

<p>This subsection of the ‘Function’ section describes regulatory mechanisms for enzymes, transporters or microbial transcription factors, and reports the components which regulate (by activation or inhibition) the reaction.<p><a href='/help/activity_regulation' target='_top'>More...</a></p>Activity regulationi

Inhibited by propargylglycine, trifluoroalanine and aminoethoxyvinylglycine.2 Publications

<p>This subsection of the ‘Function’ section describes biophysical and chemical properties, such as maximal absorption, kinetic parameters, pH dependence, redox potentials and temperature dependence.<p><a href='/help/biophysicochemical_properties' target='_top'>More...</a></p>Kineticsi

  1. KM=0.5 mM for L-cystathionine2 Publications
  2. KM=5.4 mM for homocysteine2 Publications
  3. KM=3.5 mM for cysteine2 Publications

    pH dependencei

    Optimum pH is 8.2.2 Publications

    <p>This subsection of the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section describes the metabolic pathway(s) associated with a protein.<p><a href='/help/pathway' target='_top'>More...</a></p>Pathwayi: L-cysteine biosynthesis

    This protein is involved in step 2 of the subpathway that synthesizes L-cysteine from L-homocysteine and L-serine.
    Proteins known to be involved in the 2 steps of the subpathway in this organism are:
    1. Cystathionine beta-synthase-like protein (CBSL), Cystathionine beta-synthase (CBS)
    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
    <p>This subsection of the ‘Function’ section describes the interaction between a single amino acid and another chemical entity. Priority is given to the annotation of physiological ligands.<p><a href='/help/binding' target='_top'>More...</a></p>Binding sitei62Substrate1
    Binding sitei114Substrate1
    Binding sitei119Substrate1
    Binding sitei339Substrate1

    <p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

    • calmodulin binding Source: UniProtKB-KW
    • carbon-sulfur lyase activity Source: Reactome
    • cystathionine gamma-lyase activity Source: UniProtKB
    • cystathionine gamma-synthase activity Source: GO_Central
    • homocysteine desulfhydrase activity Source: Reactome
    • identical protein binding Source: IntAct
    • L-cysteine desulfhydrase activity Source: Reactome
    • L-cystine L-cysteine-lyase (deaminating) Source: UniProtKB
    • pyridoxal phosphate binding Source: UniProtKB

    GO - Biological processi

    <p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

    Molecular functionCalmodulin-binding, Lyase
    Biological processAmino-acid biosynthesis, Cysteine biosynthesis
    LigandPyridoxal phosphate

    Enzyme and pathway databases

    BioCyc Collection of Pathway/Genome Databases

    More...
    BioCyci
    MetaCyc:HS04050-MONOMER

    BRENDA Comprehensive Enzyme Information System

    More...
    BRENDAi
    4.4.1.1 2681

    Reactome - a knowledgebase of biological pathways and processes

    More...
    Reactomei
    R-HSA-1614558 Degradation of cysteine and homocysteine
    R-HSA-1614603 Cysteine formation from homocysteine
    R-HSA-2408508 Metabolism of ingested SeMet, Sec, MeSec into H2Se

    SABIO-RK: Biochemical Reaction Kinetics Database

    More...
    SABIO-RKi
    P32929

    UniPathway: a resource for the exploration and annotation of metabolic pathways

    More...
    UniPathwayi
    UPA00136;UER00202

    <p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

    <p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
    Recommended name:
    Cystathionine gamma-lyase (EC:4.4.1.1)
    Alternative name(s):
    Cysteine-protein sulfhydrase
    Gamma-cystathionase
    <p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: ‘Name’, ‘Synonyms’, ‘Ordered locus names’ and ‘ORF names’.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
    Name:CTH
    <p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
    <p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the ‘taxonomic identifier’ or ‘taxid’.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
    <p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    <p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
    • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes_manual">proteome</a> can consist of several components. <br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 1

    Organism-specific databases

    Eukaryotic Pathogen Database Resources

    More...
    EuPathDBi
    HostDB:ENSG00000116761.11

    Human Gene Nomenclature Database

    More...
    HGNCi
    HGNC:2501 CTH

    Online Mendelian Inheritance in Man (OMIM)

    More...
    MIMi
    607657 gene

    neXtProt; the human protein knowledge platform

    More...
    neXtProti
    NX_P32929

    <p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>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

    <p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

    <p>This subsection of the ‘Pathology and Biotech’ section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>Involvement in diseasei

    Cystathioninuria (CSTNU)2 Publications
    The disease is caused by mutations affecting the gene represented in this entry.
    Disease descriptionAutosomal recessive phenotype characterized by abnormal accumulation of plasma cystathionine, leading to increased urinary excretion.
    See also OMIM:219500
    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the ‘Sequence’ section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>Natural variantiVAR_01545067T → I in CSTNU; reduces catalytic activity and affinity for pyridoxal phosphate. 2 PublicationsCorresponds to variant dbSNP:rs28941785EnsemblClinVar.1
    Natural variantiVAR_015451240Q → E in CSTNU; strongly reduces catalytic activity and affinity for pyridoxal phosphate. 2 PublicationsCorresponds to variant dbSNP:rs28941786EnsemblClinVar.1

    Keywords - Diseasei

    Disease mutation

    Organism-specific databases

    DisGeNET

    More...
    DisGeNETi
    1491

    MalaCards human disease database

    More...
    MalaCardsi
    CTH
    MIMi219500 phenotype

    Open Targets

    More...
    OpenTargetsi
    ENSG00000116761

    Orphanet; a database dedicated to information on rare diseases and orphan drugs

    More...
    Orphaneti
    212 Cystathioninuria

    The Pharmacogenetics and Pharmacogenomics Knowledge Base

    More...
    PharmGKBi
    PA27004

    Chemistry databases

    Drug and drug target database

    More...
    DrugBanki
    DB02328 2-[(3-Hydroxy-2-Methyl-5-Phosphonooxymethyl-Pyridin-4-Ylmethyl)-Imino]-5-Phosphono-Pent-3-Enoic Acid
    DB04217 L-2-amino-3-butynoic acid
    DB00151 L-Cysteine
    DB00114 Pyridoxal Phosphate

    IUPHAR/BPS Guide to PHARMACOLOGY

    More...
    GuidetoPHARMACOLOGYi
    1444

    Polymorphism and mutation databases

    BioMuta curated single-nucleotide variation and disease association database

    More...
    BioMutai
    CTH

    Domain mapping of disease mutations (DMDM)

    More...
    DMDMi
    27735163

    <p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

    Molecule processing

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the ‘PTM / Processing’ section describes the extent of a polypeptide chain in the mature protein following processing.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_00001147491 – 405Cystathionine gamma-lyaseAdd BLAST405

    Amino acid modifications

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the ‘PTM / Processing’ section specifies the position and type of each modified residue excluding <a href="http://www.uniprot.org/manual/lipid">lipids</a>, <a href="http://www.uniprot.org/manual/carbohyd">glycans</a> and <a href="http://www.uniprot.org/manual/crosslnk">protein cross-links</a>.<p><a href='/help/mod_res' target='_top'>More...</a></p>Modified residuei212N6-(pyridoxal phosphate)lysineBy similarity1

    Proteomic databases

    Encyclopedia of Proteome Dynamics

    More...
    EPDi
    P32929

    jPOST - Japan Proteome Standard Repository/Database

    More...
    jPOSTi
    P32929

    MaxQB - The MaxQuant DataBase

    More...
    MaxQBi
    P32929

    PaxDb, a database of protein abundance averages across all three domains of life

    More...
    PaxDbi
    P32929

    PeptideAtlas

    More...
    PeptideAtlasi
    P32929

    PRoteomics IDEntifications database

    More...
    PRIDEi
    P32929

    ProteomicsDB human proteome resource

    More...
    ProteomicsDBi
    54890
    54891 [P32929-2]

    PTM databases

    iPTMnet integrated resource for PTMs in systems biology context

    More...
    iPTMneti
    P32929

    Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

    More...
    PhosphoSitePlusi
    P32929

    <p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

    Gene expression databases

    Bgee dataBase for Gene Expression Evolution

    More...
    Bgeei
    ENSG00000116761 Expressed in 187 organ(s), highest expression level in liver

    CleanEx database of gene expression profiles

    More...
    CleanExi
    HS_CTH

    Genevisible search portal to normalized and curated expression data from Genevestigator

    More...
    Genevisiblei
    P32929 HS

    Organism-specific databases

    Human Protein Atlas

    More...
    HPAi
    HPA021591
    HPA023300

    <p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

    <p>This subsection of the <a href="http://www.uniprot.org/help/interaction_section">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

    Homotetramer. Interacts with CALM in a calcium-dependent manner (By similarity).By similarity

    <p>This subsection of the '<a href="http://www.uniprot.org/help/interaction_section%27">Interaction</a> section provides information about binary protein-protein interactions. The data presented in this section are a quality-filtered subset of binary interactions automatically derived from the <a href="http://www.ebi.ac.uk/intact/">IntAct database</a>. It is updated on a monthly basis. Each binary interaction is displayed on a separate line.<p><a href='/help/binary_interactions' target='_top'>More...</a></p>Binary interactionsi

    GO - Molecular functioni

    Protein-protein interaction databases

    The Biological General Repository for Interaction Datasets (BioGrid)

    More...
    BioGridi
    107873, 51 interactors

    Protein interaction database and analysis system

    More...
    IntActi
    P32929, 6 interactors

    Molecular INTeraction database

    More...
    MINTi
    P32929

    STRING: functional protein association networks

    More...
    STRINGi
    9606.ENSP00000359976

    Chemistry databases

    BindingDB database of measured binding affinities

    More...
    BindingDBi
    P32929

    <p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

    Secondary structure

    1405
    Legend: HelixTurnBeta strandPDB Structure known for this area
    Show more details

    3D structure databases

    Select the link destinations:

    Protein Data Bank Europe

    More...
    PDBei

    Protein Data Bank RCSB

    More...
    RCSB PDBi

    Protein Data Bank Japan

    More...
    PDBji
    Links Updated
    PDB entryMethodResolution (Å)ChainPositionsPDBsum
    2NMPX-ray2.60A/B/C/D1-402[»]
    3COGX-ray2.00A/B/C/D1-402[»]
    3ELPX-ray2.40A/B/C/D1-405[»]
    5EIGX-ray2.70A/B/C/D/E/F/G/H1-405[»]
    5TSUX-ray2.20A/B/C/D/E/F/G/H2-405[»]
    5TT2X-ray2.95C/D2-405[»]

    Protein Model Portal of the PSI-Nature Structural Biology Knowledgebase

    More...
    ProteinModelPortali
    P32929

    SWISS-MODEL Repository - a database of annotated 3D protein structure models

    More...
    SMRi
    P32929

    Database of comparative protein structure models

    More...
    ModBasei
    Search...

    MobiDB: a database of protein disorder and mobility annotations

    More...
    MobiDBi
    Search...

    Miscellaneous databases

    Relative evolutionary importance of amino acids within a protein sequence

    More...
    EvolutionaryTracei
    P32929

    <p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

    <p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

    Belongs to the trans-sulfuration enzymes family.Curated

    Phylogenomic databases

    evolutionary genealogy of genes: Non-supervised Orthologous Groups

    More...
    eggNOGi
    KOG0053 Eukaryota
    COG0626 LUCA

    Ensembl GeneTree

    More...
    GeneTreei
    ENSGT00390000000312

    The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

    More...
    HOGENOMi
    HOG000246415

    The HOVERGEN Database of Homologous Vertebrate Genes

    More...
    HOVERGENi
    HBG005322

    InParanoid: Eukaryotic Ortholog Groups

    More...
    InParanoidi
    P32929

    KEGG Orthology (KO)

    More...
    KOi
    K01758

    Identification of Orthologs from Complete Genome Data

    More...
    OMAi
    HPGRMTH

    Database of Orthologous Groups

    More...
    OrthoDBi
    1190548at2759

    Database for complete collections of gene phylogenies

    More...
    PhylomeDBi
    P32929

    TreeFam database of animal gene trees

    More...
    TreeFami
    TF300720

    Family and domain databases

    Conserved Domains Database

    More...
    CDDi
    cd00614 CGS_like, 1 hit

    Gene3D Structural and Functional Annotation of Protein Families

    More...
    Gene3Di
    3.40.640.10, 1 hit
    3.90.1150.10, 1 hit

    Integrated resource of protein families, domains and functional sites

    More...
    InterProi
    View protein in InterPro
    IPR000277 Cys/Met-Metab_PyrdxlP-dep_enz
    IPR015424 PyrdxlP-dep_Trfase
    IPR015422 PyrdxlP-dep_Trfase_dom1
    IPR015421 PyrdxlP-dep_Trfase_major

    The PANTHER Classification System

    More...
    PANTHERi
    PTHR11808 PTHR11808, 1 hit

    Pfam protein domain database

    More...
    Pfami
    View protein in Pfam
    PF01053 Cys_Met_Meta_PP, 1 hit

    PIRSF; a whole-protein classification database

    More...
    PIRSFi
    PIRSF001434 CGS, 1 hit

    Superfamily database of structural and functional annotation

    More...
    SUPFAMi
    SSF53383 SSF53383, 1 hit

    PROSITE; a protein domain and family database

    More...
    PROSITEi
    View protein in PROSITE
    PS00868 CYS_MET_METAB_PP, 1 hit

    <p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence_length">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>.<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequences (3)i

    <p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

    This entry describes 3 <p>This subsection of the ‘Sequence’ section lists the alternative protein sequences (isoforms) that can be generated from the same gene by a single or by the combination of up to four biological events (alternative promoter usage, alternative splicing, alternative initiation and ribosomal frameshifting). Additionally, this section gives relevant information on each alternative protein isoform.<p><a href='/help/alternative_products' target='_top'>More...</a></p> isoformsi produced by alternative splicing. AlignAdd to basket
    Isoform 1 (identifier: P32929-1) [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
    MQEKDASSQG FLPHFQHFAT QAIHVGQDPE QWTSRAVVPP ISLSTTFKQG
    60 70 80 90 100
    APGQHSGFEY SRSGNPTRNC LEKAVAALDG AKYCLAFASG LAATVTITHL
    110 120 130 140 150
    LKAGDQIICM DDVYGGTNRY FRQVASEFGL KISFVDCSKI KLLEAAITPE
    160 170 180 190 200
    TKLVWIETPT NPTQKVIDIE GCAHIVHKHG DIILVVDNTF MSPYFQRPLA
    210 220 230 240 250
    LGADISMYSA TKYMNGHSDV VMGLVSVNCE SLHNRLRFLQ NSLGAVPSPI
    260 270 280 290 300
    DCYLCNRGLK TLHVRMEKHF KNGMAVAQFL ESNPWVEKVI YPGLPSHPQH
    310 320 330 340 350
    ELVKRQCTGC TGMVTFYIKG TLQHAEIFLK NLKLFTLAES LGGFESLAEL
    360 370 380 390 400
    PAIMTHASVL KNDRDVLGIS DTLIRLSVGL EDEEDLLEDL DQALKAAHPP

    SGSHS
    Length:405
    Mass (Da):44,508
    Last modified:January 10, 2003 - v3
    <p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:i003246D7C1D16723
    GO
    Isoform 2 (identifier: P32929-2) [UniParc]FASTAAdd to basket

    The sequence of this isoform differs from the canonical sequence as follows:
         153-196: Missing.

    Show »
    Length:361
    Mass (Da):39,505
    Checksum:iD6AE74B370664D23
    GO
    Isoform 3 (identifier: P32929-3) [UniParc]FASTAAdd to basket

    The sequence of this isoform differs from the canonical sequence as follows:
         85-116: Missing.

    Show »
    Length:373
    Mass (Da):41,260
    Checksum:i0A403F56C25EC633
    GO

    Natural variant

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Natural variantiVAR_01545067T → I in CSTNU; reduces catalytic activity and affinity for pyridoxal phosphate. 2 PublicationsCorresponds to variant dbSNP:rs28941785EnsemblClinVar.1
    Natural variantiVAR_015451240Q → E in CSTNU; strongly reduces catalytic activity and affinity for pyridoxal phosphate. 2 PublicationsCorresponds to variant dbSNP:rs28941786EnsemblClinVar.1
    Natural variantiVAR_015452403S → I4 PublicationsCorresponds to variant dbSNP:rs1021737EnsemblClinVar.1

    Alternative sequence

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the ‘Sequence’ section describes the sequence of naturally occurring alternative protein isoform(s). The changes in the amino acid sequence may be due to alternative splicing, alternative promoter usage, alternative initiation, or ribosomal frameshifting. The information stored in this subsection is used to automatically construct alternative protein sequence(s) for display.<p><a href='/help/var_seq' target='_top'>More...</a></p>Alternative sequenceiVSP_04727485 – 116Missing in isoform 3. 1 PublicationAdd BLAST32
    Alternative sequenceiVSP_006306153 – 196Missing in isoform 2. 1 PublicationAdd BLAST44

    Sequence databases

    Select the link destinations:

    EMBL nucleotide sequence database

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    EMBLi

    GenBank nucleotide sequence database

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    GenBanki

    DNA Data Bank of Japan; a nucleotide sequence database

    More...
    DDBJi
    Links Updated
    S52784 mRNA Translation: AAB24700.1
    S52028 mRNA Translation: AAB24699.1
    BT006882 mRNA Translation: AAP35528.1
    AK303946 mRNA Translation: BAG64874.1
    AK223376 mRNA Translation: BAD97096.1
    AL354872 Genomic DNA No translation available.
    CH471059 Genomic DNA Translation: EAX06450.1
    BC015807 mRNA Translation: AAH15807.1

    The Consensus CDS (CCDS) project

    More...
    CCDSi
    CCDS53333.1 [P32929-3]
    CCDS650.1 [P32929-1]
    CCDS651.1 [P32929-2]

    Protein sequence database of the Protein Information Resource

    More...
    PIRi
    JC1362

    NCBI Reference Sequences

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    RefSeqi
    NP_001177392.1, NM_001190463.1 [P32929-3]
    NP_001893.2, NM_001902.5 [P32929-1]
    NP_714964.2, NM_153742.4 [P32929-2]

    UniGene gene-oriented nucleotide sequence clusters

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    UniGenei
    Hs.19904

    Genome annotation databases

    Ensembl eukaryotic genome annotation project

    More...
    Ensembli
    ENST00000346806; ENSP00000311554; ENSG00000116761 [P32929-2]
    ENST00000370938; ENSP00000359976; ENSG00000116761 [P32929-1]
    ENST00000411986; ENSP00000413407; ENSG00000116761 [P32929-3]

    Database of genes from NCBI RefSeq genomes

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    GeneIDi
    1491

    KEGG: Kyoto Encyclopedia of Genes and Genomes

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    KEGGi
    hsa:1491

    UCSC genome browser

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    UCSCi
    uc001dfd.4 human [P32929-1]

    Keywords - Coding sequence diversityi

    Alternative splicing, Polymorphism

    <p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

    <p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBLi
    GenBanki
    DDBJi
    Links Updated
    S52784 mRNA Translation: AAB24700.1
    S52028 mRNA Translation: AAB24699.1
    BT006882 mRNA Translation: AAP35528.1
    AK303946 mRNA Translation: BAG64874.1
    AK223376 mRNA Translation: BAD97096.1
    AL354872 Genomic DNA No translation available.
    CH471059 Genomic DNA Translation: EAX06450.1
    BC015807 mRNA Translation: AAH15807.1
    CCDSiCCDS53333.1 [P32929-3]
    CCDS650.1 [P32929-1]
    CCDS651.1 [P32929-2]
    PIRiJC1362
    RefSeqiNP_001177392.1, NM_001190463.1 [P32929-3]
    NP_001893.2, NM_001902.5 [P32929-1]
    NP_714964.2, NM_153742.4 [P32929-2]
    UniGeneiHs.19904

    3D structure databases

    Select the link destinations:
    PDBei
    RCSB PDBi
    PDBji
    Links Updated
    PDB entryMethodResolution (Å)ChainPositionsPDBsum
    2NMPX-ray2.60A/B/C/D1-402[»]
    3COGX-ray2.00A/B/C/D1-402[»]
    3ELPX-ray2.40A/B/C/D1-405[»]
    5EIGX-ray2.70A/B/C/D/E/F/G/H1-405[»]
    5TSUX-ray2.20A/B/C/D/E/F/G/H2-405[»]
    5TT2X-ray2.95C/D2-405[»]
    ProteinModelPortaliP32929
    SMRiP32929
    ModBaseiSearch...
    MobiDBiSearch...

    Protein-protein interaction databases

    BioGridi107873, 51 interactors
    IntActiP32929, 6 interactors
    MINTiP32929
    STRINGi9606.ENSP00000359976

    Chemistry databases

    BindingDBiP32929
    DrugBankiDB02328 2-[(3-Hydroxy-2-Methyl-5-Phosphonooxymethyl-Pyridin-4-Ylmethyl)-Imino]-5-Phosphono-Pent-3-Enoic Acid
    DB04217 L-2-amino-3-butynoic acid
    DB00151 L-Cysteine
    DB00114 Pyridoxal Phosphate
    GuidetoPHARMACOLOGYi1444

    PTM databases

    iPTMnetiP32929
    PhosphoSitePlusiP32929

    Polymorphism and mutation databases

    BioMutaiCTH
    DMDMi27735163

    Proteomic databases

    EPDiP32929
    jPOSTiP32929
    MaxQBiP32929
    PaxDbiP32929
    PeptideAtlasiP32929
    PRIDEiP32929
    ProteomicsDBi54890
    54891 [P32929-2]

    Protocols and materials databases

    The DNASU plasmid repository

    More...
    DNASUi
    1491
    Structural Biology KnowledgebaseSearch...

    Genome annotation databases

    EnsembliENST00000346806; ENSP00000311554; ENSG00000116761 [P32929-2]
    ENST00000370938; ENSP00000359976; ENSG00000116761 [P32929-1]
    ENST00000411986; ENSP00000413407; ENSG00000116761 [P32929-3]
    GeneIDi1491
    KEGGihsa:1491
    UCSCiuc001dfd.4 human [P32929-1]

    Organism-specific databases

    Comparative Toxicogenomics Database

    More...
    CTDi
    1491
    DisGeNETi1491
    EuPathDBiHostDB:ENSG00000116761.11

    GeneCards: human genes, protein and diseases

    More...
    GeneCardsi
    CTH
    HGNCiHGNC:2501 CTH
    HPAiHPA021591
    HPA023300
    MalaCardsiCTH
    MIMi219500 phenotype
    607657 gene
    neXtProtiNX_P32929
    OpenTargetsiENSG00000116761
    Orphaneti212 Cystathioninuria
    PharmGKBiPA27004

    GenAtlas: human gene database

    More...
    GenAtlasi
    Search...

    Phylogenomic databases

    eggNOGiKOG0053 Eukaryota
    COG0626 LUCA
    GeneTreeiENSGT00390000000312
    HOGENOMiHOG000246415
    HOVERGENiHBG005322
    InParanoidiP32929
    KOiK01758
    OMAiHPGRMTH
    OrthoDBi1190548at2759
    PhylomeDBiP32929
    TreeFamiTF300720

    Enzyme and pathway databases

    UniPathwayi
    UPA00136;UER00202

    BioCyciMetaCyc:HS04050-MONOMER
    BRENDAi4.4.1.1 2681
    ReactomeiR-HSA-1614558 Degradation of cysteine and homocysteine
    R-HSA-1614603 Cysteine formation from homocysteine
    R-HSA-2408508 Metabolism of ingested SeMet, Sec, MeSec into H2Se
    SABIO-RKiP32929

    Miscellaneous databases

    ChiTaRS: a database of human, mouse and fruit fly chimeric transcripts and RNA-sequencing data

    More...
    ChiTaRSi
    CTH human
    EvolutionaryTraceiP32929

    Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

    More...
    GenomeRNAii
    1491

    Protein Ontology

    More...
    PROi
    PR:P32929

    The Stanford Online Universal Resource for Clones and ESTs

    More...
    SOURCEi
    Search...

    Gene expression databases

    BgeeiENSG00000116761 Expressed in 187 organ(s), highest expression level in liver
    CleanExiHS_CTH
    GenevisibleiP32929 HS

    Family and domain databases

    CDDicd00614 CGS_like, 1 hit
    Gene3Di3.40.640.10, 1 hit
    3.90.1150.10, 1 hit
    InterProiView protein in InterPro
    IPR000277 Cys/Met-Metab_PyrdxlP-dep_enz
    IPR015424 PyrdxlP-dep_Trfase
    IPR015422 PyrdxlP-dep_Trfase_dom1
    IPR015421 PyrdxlP-dep_Trfase_major
    PANTHERiPTHR11808 PTHR11808, 1 hit
    PfamiView protein in Pfam
    PF01053 Cys_Met_Meta_PP, 1 hit
    PIRSFiPIRSF001434 CGS, 1 hit
    SUPFAMiSSF53383 SSF53383, 1 hit
    PROSITEiView protein in PROSITE
    PS00868 CYS_MET_METAB_PP, 1 hit

    ProtoNet; Automatic hierarchical classification of proteins

    More...
    ProtoNeti
    Search...

    <p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

    <p>This subsection of the ‘Entry information’ section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiCGL_HUMAN
    <p>This subsection of the ‘Entry information’ section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called ‘Primary (citable) accession number’.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: P32929
    Secondary accession number(s): B4E1R2
    , E9PDV0, Q53FB3, Q53Y79, Q9H4W7, Q9H4W8
    <p>This subsection of the ‘Entry information’ section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification (‘Last modified’). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 1, 1993
    Last sequence update: January 10, 2003
    Last modified: January 16, 2019
    This is version 188 of the entry and version 3 of the sequence. See complete history.
    <p>This subsection of the ‘Entry information’ section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>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.

    <p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

    Keywords - Technical termi

    3D-structure, Complete proteome, Reference proteome

    Documents

    1. SIMILARITY comments
      Index of protein domains and families
    2. Human entries with polymorphisms or disease mutations
      List of human entries with polymorphisms or disease mutations
    3. Human polymorphisms and disease mutations
      Index of human polymorphisms and disease mutations
    4. MIM cross-references
      Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
    5. PATHWAY comments
      Index of metabolic and biosynthesis pathways
    6. PDB cross-references
      Index of Protein Data Bank (PDB) cross-references
    7. Human chromosome 1
      Human chromosome 1: entries, gene names and cross-references to MIM
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