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Q01634

- IDUA_CANFA

UniProt

Q01634 - IDUA_CANFA

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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

Functioni

Catalytic activityi

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

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Binding sitei90 – 901Substrate By similarity
Binding sitei180 – 1801Substrate By similarity
Active sitei181 – 1811Proton donor By similarity
Binding sitei263 – 2631Substrate By similarity
Active sitei298 – 2981Nucleophile By similarity
Binding sitei348 – 3481Substrate By similarity
Binding sitei362 – 3621Substrate By 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
Complete GO annotation...

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
Complete GO annotation...

Keywords - Cellular componenti

Lysosome

Pathology & Biotechi

Involvement in diseasei

Defects in IDUA are the cause of mucopolysaccharidosis type I (MPS I).1 Publication

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...) Reviewed prediction
Glycosylationi189 – 1891N-linked (GlcNAc...) Reviewed prediction
Glycosylationi242 – 2421N-linked (GlcNAc...) Reviewed prediction
Glycosylationi335 – 3351N-linked (GlcNAc...) Reviewed prediction
Glycosylationi371 – 3711N-linked (GlcNAc...) Reviewed prediction
Glycosylationi414 – 4141N-linked (GlcNAc...) Reviewed prediction
Disulfide bondi540 ↔ 576 By 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.

Family & Domainsi

Region

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

Sequence similaritiesi

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

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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: December 11, 2013
This is version 79 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

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