Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Basket 0
(max 400 entries)x

Your basket is currently empty.

Select item(s) and click on "Add to basket" to create your own collection here
(400 entries max)

Q01634

- IDUA_CANFA

UniProt

Q01634 - IDUA_CANFA

Protein

Alpha-L-iduronidase

Gene

IDUA

Organism
Canis familiaris (Dog) (Canis lupus familiaris)
Status
Reviewed - Annotation score: 4 out of 5- Experimental evidence at protein leveli
    • BLAST
    • Align
    • Format
    • Add to basket
    • History
      Entry version 80 (01 Oct 2014)
      Sequence version 1 (01 Feb 1994)
      Previous versions | rss
    • Help video
    • Feedback
    • Comment

    Functioni

    Catalytic activityi

    Hydrolysis of unsulfated alpha-L-iduronosidic linkages in dermatan sulfate.1 Publication

    Sites

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Binding sitei90 – 901SubstrateBy similarity
    Binding sitei180 – 1801SubstrateBy similarity
    Active sitei181 – 1811Proton donorPROSITE-ProRule annotation
    Binding sitei263 – 2631SubstrateBy similarity
    Active sitei298 – 2981NucleophileBy similarity
    Binding sitei348 – 3481SubstrateBy similarity
    Binding sitei362 – 3621SubstrateBy similarity

    GO - Molecular functioni

    1. L-iduronidase activity Source: UniProtKB

    GO - Biological processi

    1. carbohydrate metabolic process Source: InterPro
    2. dermatan sulfate catabolic process Source: UniProtKB

    Keywords - Molecular functioni

    Glycosidase, Hydrolase

    Protein family/group databases

    CAZyiGH39. Glycoside Hydrolase Family 39.

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Alpha-L-iduronidase (EC:3.2.1.76)
    Gene namesi
    Name:IDUA
    OrganismiCanis familiaris (Dog) (Canis lupus familiaris)
    Taxonomic identifieri9615 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaLaurasiatheriaCarnivoraCaniformiaCanidaeCanis
    ProteomesiUP000002254: Unplaced

    Subcellular locationi

    Lysosome By similarity

    GO - Cellular componenti

    1. lysosome Source: UniProtKB-SubCell

    Keywords - Cellular componenti

    Lysosome

    Pathology & Biotechi

    Involvement in diseasei

    Defects in IDUA are the cause of mucopolysaccharidosis type I (MPS I).

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Signal peptidei1 – 2525Add
    BLAST
    Chaini26 – 655630Alpha-L-iduronidasePRO_0000012199Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Glycosylationi109 – 1091N-linked (GlcNAc...)Sequence Analysis
    Glycosylationi189 – 1891N-linked (GlcNAc...)Sequence Analysis
    Glycosylationi242 – 2421N-linked (GlcNAc...)Sequence Analysis
    Glycosylationi335 – 3351N-linked (GlcNAc...)Sequence Analysis
    Glycosylationi371 – 3711N-linked (GlcNAc...)Sequence Analysis
    Glycosylationi414 – 4141N-linked (GlcNAc...)Sequence Analysis
    Disulfide bondi540 ↔ 576By similarity

    Post-translational modificationi

    A smaller 63 kDa protein probably arises from IDUA protein by proteolytic cleavage.
    N-glycosylation contributes to substrate binding and is required for full enzymatic activity.By similarity

    Keywords - PTMi

    Disulfide bond, Glycoprotein

    Expressioni

    Tissue specificityi

    Detected in testis (at protein level). Expressed ubiquitously.1 Publication

    Interactioni

    Subunit structurei

    Monomer.By similarity

    Protein-protein interaction databases

    STRINGi9615.ENSCAFP00000024558.

    Structurei

    3D structure databases

    ProteinModelPortaliQ01634.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Region

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Regioni304 – 3052Substrate bindingBy similarity

    Sequence similaritiesi

    Belongs to the glycosyl hydrolase 39 family.Curated

    Keywords - Domaini

    Signal

    Phylogenomic databases

    eggNOGiNOG74108.
    HOVERGENiHBG006121.

    Family and domain databases

    Gene3Di3.20.20.80. 1 hit.
    InterProiIPR003961. Fibronectin_type3.
    IPR000514. Glyco_hydro_39.
    IPR013781. Glyco_hydro_catalytic_dom.
    IPR017853. Glycoside_hydrolase_SF.
    [Graphical view]
    PfamiPF01229. Glyco_hydro_39. 1 hit.
    [Graphical view]
    PRINTSiPR00745. GLHYDRLASE39.
    SUPFAMiSSF49265. SSF49265. 1 hit.
    SSF51445. SSF51445. 1 hit.
    PROSITEiPS01027. GLYCOSYL_HYDROL_F39. 1 hit.
    [Graphical view]

    Sequencei

    Sequence statusi: Complete.

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

    Q01634-1 [UniParc]FASTAAdd to Basket

    « Hide

    MRPPGPRAPG LALLAALLAA PRALAEAPHL VLVDAARALR PLRPFWRSTG    50
    FCPPLPHSQA DRYDLSWDQQ LNLAYVGAVP HGGIEQVRTH WLLELITARE 100
    SAGQGLSYNF THLDGYLDLL RENQLLPGFE LMGSPSQRFT DFEDKRQVLA 150
    WKELVSLLAR RYIGRYGLSY VSKWNFETWN EPDHHDFDNV TMTLQGFLNY 200
    YDACSEGLRA ASPALRFGGP GDSFHPWPRS PLCWGLLEHC HNGTNFFTGE 250
    LGVRLDYISL HKKGAGSSIY ILEQEQATVQ QIRRLFPKFA DTPVYNDEAD 300
    PLVGWALPQP WRADVTYAAM VVKVVAQHQN PPRANGSAAL RPALLSNDNA 350
    FLSFHPHPFT QRTLTARFQV NDTEPPHVQL LRKPVLTAMA LLALLDGRQL 400
    WAEVSRGGTV LDSNHTVGVL ASAHLPAGPR DAWRATVLLY ASDDTRAHAA 450
    RAVPVTLRLL GVPRGPGLVY VTLALDNPRC SPHGEWQRLG RPVFPTAEEF 500
    RRMRAAEDPV AEAPRPFPAS GRLTLSVELR LPSLLLLHVC ARPEKPPGPV 550
    TRLRALPLTR GQVLLVWSDE RVGSKCLWTY EIQFSADGEV YTPISRKPST 600
    FNLFVFSPES AVTSGSYRVR AVDYWARPGP FSTRVHYVEV PAPSGPPRPS 650
    DCERC 655
    Length:655
    Mass (Da):72,939
    Last modified:February 1, 1994 - v1
    Checksum:i294A56333FE7BBC9
    GO

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    L01058 Genomic DNA. No translation available.
    L01059 Genomic DNA. No translation available.
    L01060 Genomic DNA. No translation available.
    L01061 Genomic DNA. No translation available.
    L01065 Genomic DNA. Translation: AAA51456.1.
    M81893 mRNA. Translation: AAA51455.1.
    PIRiA42420.

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    L01058 Genomic DNA. No translation available.
    L01059 Genomic DNA. No translation available.
    L01060 Genomic DNA. No translation available.
    L01061 Genomic DNA. No translation available.
    L01065 Genomic DNA. Translation: AAA51456.1 .
    M81893 mRNA. Translation: AAA51455.1 .
    PIRi A42420.

    3D structure databases

    ProteinModelPortali Q01634.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    STRINGi 9615.ENSCAFP00000024558.

    Protein family/group databases

    CAZyi GH39. Glycoside Hydrolase Family 39.

    Protocols and materials databases

    Structural Biology Knowledgebase Search...

    Phylogenomic databases

    eggNOGi NOG74108.
    HOVERGENi HBG006121.

    Family and domain databases

    Gene3Di 3.20.20.80. 1 hit.
    InterProi IPR003961. Fibronectin_type3.
    IPR000514. Glyco_hydro_39.
    IPR013781. Glyco_hydro_catalytic_dom.
    IPR017853. Glycoside_hydrolase_SF.
    [Graphical view ]
    Pfami PF01229. Glyco_hydro_39. 1 hit.
    [Graphical view ]
    PRINTSi PR00745. GLHYDRLASE39.
    SUPFAMi SSF49265. SSF49265. 1 hit.
    SSF51445. SSF51445. 1 hit.
    PROSITEi PS01027. GLYCOSYL_HYDROL_F39. 1 hit.
    [Graphical view ]
    ProtoNeti Search...

    Publicationsi

    1. "Cloning and characterization of cDNA encoding canine alpha-L-iduronidase. mRNA deficiency in mucopolysaccharidosis I dog."
      Stoltzfus L.J., Sosa-Pineda B., Moskowitz S.M., Menon K.P., Dlott B., Hooper L., Teplow D.B., Shull R.M., Neufeld E.F.
      J. Biol. Chem. 267:6570-6575(1992) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA / MRNA], PARTIAL PROTEIN SEQUENCE, CATALYTIC ACTIVITY, TISSUE SPECIFICITY.
      Tissue: Testis.
    2. "Architecture of the canine IDUA gene and mutation underlying canine mucopolysaccharidosis I."
      Menon K.P., Tieu P.T., Neufeld E.F.
      Genomics 14:763-768(1992) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA], ROLE IN DISEASE.
      Tissue: Fibroblast and Testis.

    Entry informationi

    Entry nameiIDUA_CANFA
    AccessioniPrimary (citable) accession number: Q01634
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: February 1, 1994
    Last sequence update: February 1, 1994
    Last modified: October 1, 2014
    This is version 80 of the entry and version 1 of the sequence. [Complete history]
    Entry statusiReviewed (UniProtKB/Swiss-Prot)
    Annotation programChordata Protein Annotation Program

    Miscellaneousi

    Keywords - Technical termi

    Complete proteome, Direct protein sequencing, Reference proteome

    Documents

    1. Glycosyl hydrolases
      Classification of glycosyl hydrolase families and list of entries
    2. SIMILARITY comments
      Index of protein domains and families

    External Data

    Dasty 3