Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.

Q01634

- IDUA_CANFA

UniProt

Q01634 - IDUA_CANFA

(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)

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 – 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
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-KW
Complete GO annotation...

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.
InParanoidiQ01634.

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 [UniParc]FASTAAdd to Basket

« Hide

        10         20         30         40         50
MRPPGPRAPG LALLAALLAA PRALAEAPHL VLVDAARALR PLRPFWRSTG
60 70 80 90 100
FCPPLPHSQA DRYDLSWDQQ LNLAYVGAVP HGGIEQVRTH WLLELITARE
110 120 130 140 150
SAGQGLSYNF THLDGYLDLL RENQLLPGFE LMGSPSQRFT DFEDKRQVLA
160 170 180 190 200
WKELVSLLAR RYIGRYGLSY VSKWNFETWN EPDHHDFDNV TMTLQGFLNY
210 220 230 240 250
YDACSEGLRA ASPALRFGGP GDSFHPWPRS PLCWGLLEHC HNGTNFFTGE
260 270 280 290 300
LGVRLDYISL HKKGAGSSIY ILEQEQATVQ QIRRLFPKFA DTPVYNDEAD
310 320 330 340 350
PLVGWALPQP WRADVTYAAM VVKVVAQHQN PPRANGSAAL RPALLSNDNA
360 370 380 390 400
FLSFHPHPFT QRTLTARFQV NDTEPPHVQL LRKPVLTAMA LLALLDGRQL
410 420 430 440 450
WAEVSRGGTV LDSNHTVGVL ASAHLPAGPR DAWRATVLLY ASDDTRAHAA
460 470 480 490 500
RAVPVTLRLL GVPRGPGLVY VTLALDNPRC SPHGEWQRLG RPVFPTAEEF
510 520 530 540 550
RRMRAAEDPV AEAPRPFPAS GRLTLSVELR LPSLLLLHVC ARPEKPPGPV
560 570 580 590 600
TRLRALPLTR GQVLLVWSDE RVGSKCLWTY EIQFSADGEV YTPISRKPST
610 620 630 640 650
FNLFVFSPES AVTSGSYRVR AVDYWARPGP FSTRVHYVEV PAPSGPPRPS

DCERC
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.
InParanoidi Q01634.

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 29, 2014
This is version 81 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