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Protein

Carbohydrate sulfotransferase 8

Gene

CHST8

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Catalyzes the transfer of sulfate to position 4 of non-reducing N-acetylgalactosamine (GalNAc) residues in both N-glycans and O-glycans. Required for biosynthesis of glycoprotein hormones lutropin and thyrotropin, by mediating sulfation of their carbohydrate structures. Only active against terminal GalNAcbeta1,GalNAcbeta. Not active toward chondroitin.2 Publications

Kineticsi

  1. KM=10 µM for carbonic anhydrase VI1 Publication

    pH dependencei

    Optimum pH is 7.2.1 Publication

    Regions

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Nucleotide bindingi198 – 2047PAPSBy similarity
    Nucleotide bindingi258 – 2669PAPSBy similarity

    GO - Molecular functioni

    • N-acetylgalactosamine 4-O-sulfotransferase activity Source: UniProtKB

    GO - Biological processi

    • carbohydrate biosynthetic process Source: InterPro
    • central nervous system development Source: UniProtKB
    • hormone biosynthetic process Source: UniProtKB
    • proteoglycan biosynthetic process Source: UniProtKB
    • sulfur compound metabolic process Source: UniProtKB
    Complete GO annotation...

    Keywords - Molecular functioni

    Transferase

    Keywords - Biological processi

    Carbohydrate metabolism

    Enzyme and pathway databases

    ReactomeiR-HSA-975578. Reactions specific to the complex N-glycan synthesis pathway.
    SABIO-RKQ9H2A9.

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Carbohydrate sulfotransferase 8 (EC:2.8.2.-)
    Alternative name(s):
    GalNAc-4-O-sulfotransferase 1
    Short name:
    GalNAc-4-ST1
    Short name:
    GalNAc4ST-1
    N-acetylgalactosamine-4-O-sulfotransferase 1
    Gene namesi
    Name:CHST8
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    Proteomesi
    • UP000005640 Componenti: Chromosome 19

    Organism-specific databases

    HGNCiHGNC:15993. CHST8.

    Subcellular locationi

    Topology

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Topological domaini1 – 1010CytoplasmicSequence analysis
    Transmembranei11 – 3121Helical; Signal-anchor for type II membrane proteinSequence analysisAdd
    BLAST
    Topological domaini32 – 424393LumenalSequence analysisAdd
    BLAST

    GO - Cellular componenti

    • Golgi membrane Source: Reactome
    • integral component of membrane Source: UniProtKB
    Complete GO annotation...

    Keywords - Cellular componenti

    Golgi apparatus, Membrane

    Pathology & Biotechi

    Involvement in diseasei

    Peeling skin syndrome 3 (PSS3)1 Publication
    The disease is caused by mutations affecting the gene represented in this entry.
    Disease descriptionA form of peeling skin syndrome, a genodermatosis characterized by generalized, continuous shedding of the outer layers of the epidermis. Two main PSS subtypes have been suggested. Patients with non-inflammatory PSS (type A) manifest white scaling, with painless and easy removal of the skin, irritation when in contact with water, dust and sand, and no history of erythema, pruritis or atopy. Inflammatory PSS (type B) is associated with generalized erythema, pruritus and atopy. It is an ichthyosiform erythroderma characterized by lifelong patchy peeling of the entire skin with onset at birth or shortly after. Several patients have been reported with high IgE levels. PSS3 is characterized by generalized white scaling occurring over the upper and lower extremities. Symptoms start during the second half of the first decade of life.
    See also OMIM:616265
    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti77 – 771R → W in PSS3; results in decreased enzyme activity; the mutant protein shows reduced glycosylation. 1 Publication
    Corresponds to variant rs149660944 [ dbSNP | Ensembl ].
    VAR_067723

    Keywords - Diseasei

    Disease mutation

    Organism-specific databases

    MalaCardsiCHST8.
    MIMi616265. phenotype.
    Orphaneti263548. Peeling skin syndrome type A.
    PharmGKBiPA26508.

    Polymorphism and mutation databases

    BioMutaiCHST8.
    DMDMi61212124.

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Chaini1 – 424424Carbohydrate sulfotransferase 8PRO_0000189653Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Glycosylationi128 – 1281N-linked (GlcNAc...)Sequence analysis
    Glycosylationi294 – 2941N-linked (GlcNAc...)Sequence analysis
    Glycosylationi367 – 3671N-linked (GlcNAc...)Sequence analysis
    Glycosylationi415 – 4151N-linked (GlcNAc...)Sequence analysis

    Keywords - PTMi

    Glycoprotein

    Proteomic databases

    EPDiQ9H2A9.
    PaxDbiQ9H2A9.
    PeptideAtlasiQ9H2A9.
    PRIDEiQ9H2A9.
    TopDownProteomicsiQ9H2A9.

    PTM databases

    iPTMnetiQ9H2A9.
    PhosphoSiteiQ9H2A9.

    Expressioni

    Tissue specificityi

    Predominantly expressed in pituitary gland. In brain, it is expressed in pituitary gland, cerebellum, medulla oblongata, pons, thalamus and spinal cord. Expressed in the epidermis. Expressed at lower level in lung, spleen, adrenal gland, placenta, prostate, testis, mammary gland and trachea.4 Publications

    Inductioni

    Down-regulated (17-fold) in prion-infected cells.1 Publication

    Gene expression databases

    BgeeiENSG00000124302.
    CleanExiHS_CHST8.
    ExpressionAtlasiQ9H2A9. baseline and differential.
    GenevisibleiQ9H2A9. HS.

    Organism-specific databases

    HPAiHPA016004.

    Interactioni

    Protein-protein interaction databases

    BioGridi122148. 33 interactions.
    STRINGi9606.ENSP00000262622.

    Structurei

    3D structure databases

    ProteinModelPortaliQ9H2A9.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Sequence similaritiesi

    Belongs to the sulfotransferase 2 family.Curated

    Keywords - Domaini

    Signal-anchor, Transmembrane, Transmembrane helix

    Phylogenomic databases

    eggNOGiKOG4651. Eukaryota.
    ENOG4111GJR. LUCA.
    GeneTreeiENSGT00760000119214.
    HOGENOMiHOG000231801.
    HOVERGENiHBG050950.
    InParanoidiQ9H2A9.
    KOiK09672.
    OMAiSTADIQH.
    OrthoDBiEOG091G0FZO.
    PhylomeDBiQ9H2A9.
    TreeFamiTF325581.

    Family and domain databases

    InterProiIPR018011. Carb_sulfotransferase-rel.
    IPR005331. Sulfotransferase.
    [Graphical view]
    PANTHERiPTHR12137. PTHR12137. 1 hit.
    PfamiPF03567. Sulfotransfer_2. 1 hit.
    [Graphical view]

    Sequencei

    Sequence statusi: Complete.

    Q9H2A9-1 [UniParc]FASTAAdd to basket

    « Hide

            10         20         30         40         50
    MTLRPGTMRL ACMFSSILLF GAAGLLLFIS LQDPTELAPQ QVPGIKFNIR
    60 70 80 90 100
    PRQPHHDLPP GGSQDGDLKE PTERVTRDLS SGAPRGRNLP APDQPQPPLQ
    110 120 130 140 150
    RGTRLRLRQR RRRLLIKKMP AAATIPANSS DAPFIRPGPG TLDGRWVSLH
    160 170 180 190 200
    RSQQERKRVM QEACAKYRAS SSRRAVTPRH VSRIFVEDRH RVLYCEVPKA
    210 220 230 240 250
    GCSNWKRVLM VLAGLASSTA DIQHNTVHYG SALKRLDTFD RQGILHRLST
    260 270 280 290 300
    YTKMLFVREP FERLVSAFRD KFEHPNSYYH PVFGKAILAR YRANASREAL
    310 320 330 340 350
    RTGSGVRFPE FVQYLLDVHR PVGMDIHWDH VSRLCSPCLI DYDFVGKFES
    360 370 380 390 400
    MEDDANFFLS LIRAPRNLTF PRFKDRHSQE ARTTARIAHQ YFAQLSALQR
    410 420
    QRTYDFYYMD YLMFNYSKPF ADLY
    Length:424
    Mass (Da):48,834
    Last modified:March 15, 2005 - v2
    Checksum:iD4CEF1E7287D6102
    GO

    Experimental Info

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Sequence conflicti326 – 3261I → T in AAG39444 (PubMed:10988300).Curated

    Natural variant

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti77 – 771R → W in PSS3; results in decreased enzyme activity; the mutant protein shows reduced glycosylation. 1 Publication
    Corresponds to variant rs149660944 [ dbSNP | Ensembl ].
    VAR_067723
    Natural varianti247 – 2471R → H in a colorectal cancer sample; somatic mutation. 1 Publication
    VAR_036538

    Sequence databases

    Select the link destinations:
    EMBLi
    GenBanki
    DDBJi
    Links Updated
    AF300612 mRNA. Translation: AAG39444.1.
    AB047801 mRNA. Translation: BAB19806.1.
    AF305781 mRNA. Translation: AAL09373.1.
    BC011380 mRNA. Translation: AAH11380.1.
    BC014250 mRNA. Translation: AAH14250.1.
    BC018723 mRNA. Translation: AAH18723.1.
    CCDSiCCDS12433.1.
    RefSeqiNP_001121367.1. NM_001127895.1.
    NP_001121368.1. NM_001127896.1.
    NP_071912.2. NM_022467.3.
    XP_011525524.1. XM_011527222.1.
    XP_011525526.1. XM_011527224.1.
    UniGeneiHs.165724.

    Genome annotation databases

    EnsembliENST00000262622; ENSP00000262622; ENSG00000124302.
    ENST00000434302; ENSP00000392604; ENSG00000124302.
    ENST00000438847; ENSP00000393879; ENSG00000124302.
    GeneIDi64377.
    KEGGihsa:64377.
    UCSCiuc002nus.5. human.

    Keywords - Coding sequence diversityi

    Polymorphism

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBLi
    GenBanki
    DDBJi
    Links Updated
    AF300612 mRNA. Translation: AAG39444.1.
    AB047801 mRNA. Translation: BAB19806.1.
    AF305781 mRNA. Translation: AAL09373.1.
    BC011380 mRNA. Translation: AAH11380.1.
    BC014250 mRNA. Translation: AAH14250.1.
    BC018723 mRNA. Translation: AAH18723.1.
    CCDSiCCDS12433.1.
    RefSeqiNP_001121367.1. NM_001127895.1.
    NP_001121368.1. NM_001127896.1.
    NP_071912.2. NM_022467.3.
    XP_011525524.1. XM_011527222.1.
    XP_011525526.1. XM_011527224.1.
    UniGeneiHs.165724.

    3D structure databases

    ProteinModelPortaliQ9H2A9.
    ModBaseiSearch...
    MobiDBiSearch...

    Protein-protein interaction databases

    BioGridi122148. 33 interactions.
    STRINGi9606.ENSP00000262622.

    PTM databases

    iPTMnetiQ9H2A9.
    PhosphoSiteiQ9H2A9.

    Polymorphism and mutation databases

    BioMutaiCHST8.
    DMDMi61212124.

    Proteomic databases

    EPDiQ9H2A9.
    PaxDbiQ9H2A9.
    PeptideAtlasiQ9H2A9.
    PRIDEiQ9H2A9.
    TopDownProteomicsiQ9H2A9.

    Protocols and materials databases

    DNASUi64377.
    Structural Biology KnowledgebaseSearch...

    Genome annotation databases

    EnsembliENST00000262622; ENSP00000262622; ENSG00000124302.
    ENST00000434302; ENSP00000392604; ENSG00000124302.
    ENST00000438847; ENSP00000393879; ENSG00000124302.
    GeneIDi64377.
    KEGGihsa:64377.
    UCSCiuc002nus.5. human.

    Organism-specific databases

    CTDi64377.
    GeneCardsiCHST8.
    H-InvDBHIX0137482.
    HGNCiHGNC:15993. CHST8.
    HPAiHPA016004.
    MalaCardsiCHST8.
    MIMi610190. gene.
    616265. phenotype.
    neXtProtiNX_Q9H2A9.
    Orphaneti263548. Peeling skin syndrome type A.
    PharmGKBiPA26508.
    GenAtlasiSearch...

    Phylogenomic databases

    eggNOGiKOG4651. Eukaryota.
    ENOG4111GJR. LUCA.
    GeneTreeiENSGT00760000119214.
    HOGENOMiHOG000231801.
    HOVERGENiHBG050950.
    InParanoidiQ9H2A9.
    KOiK09672.
    OMAiSTADIQH.
    OrthoDBiEOG091G0FZO.
    PhylomeDBiQ9H2A9.
    TreeFamiTF325581.

    Enzyme and pathway databases

    ReactomeiR-HSA-975578. Reactions specific to the complex N-glycan synthesis pathway.
    SABIO-RKQ9H2A9.

    Miscellaneous databases

    GenomeRNAii64377.
    PROiQ9H2A9.
    SOURCEiSearch...

    Gene expression databases

    BgeeiENSG00000124302.
    CleanExiHS_CHST8.
    ExpressionAtlasiQ9H2A9. baseline and differential.
    GenevisibleiQ9H2A9. HS.

    Family and domain databases

    InterProiIPR018011. Carb_sulfotransferase-rel.
    IPR005331. Sulfotransferase.
    [Graphical view]
    PANTHERiPTHR12137. PTHR12137. 1 hit.
    PfamiPF03567. Sulfotransfer_2. 1 hit.
    [Graphical view]
    ProtoNetiSearch...

    Entry informationi

    Entry nameiCHST8_HUMAN
    AccessioniPrimary (citable) accession number: Q9H2A9
    Secondary accession number(s): Q9H3N2
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: March 15, 2005
    Last sequence update: March 15, 2005
    Last modified: September 7, 2016
    This is version 108 of the entry and version 2 of the sequence. [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

    Caution

    PubMed:10988300 reports the possible existence of a secreted isoform starting at Met-119. However, they do not provide any experimental evidence.Curated

    Keywords - Technical termi

    Complete proteome, Reference proteome

    Documents

    1. Human chromosome 19
      Human chromosome 19: entries, gene names and cross-references to MIM
    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. SIMILARITY comments
      Index of protein domains and families

    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 one 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.