P35475 (IDUA_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

Recommended name:
Alpha-L-iduronidase
EC=3.2.1.76

Gene names

Name:

IDUA

Organism

Homo sapiens (Human)

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo

Protein attributes

Sequence length	653 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is further processed into a mature form.
Protein existence	Evidence at protein level

General annotation (Comments)

Catalytic activity	Hydrolysis of unsulfated alpha-L-iduronosidic linkages in dermatan sulfate.
Subunit structure	Monomer Probable.
Subcellular location	Lysosome.
Tissue specificity	Ubiquitous.
Involvement in disease	Defects in IDUA are the cause of mucopolysaccharidosis type 1H (MPS1H) [MIM:607014]; also known as Hurler syndrome. MPS1H is a severe form of mucopolysaccharidosis type 1, a rare lysosomal storage disease characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. Patients with MPS1H usually present, within the first year of life, a combination of hepatosplenomegaly, skeletal deformities, corneal clouding and severe mental retardation. Obstructive airways disease, respiratory infection and cardiac complications usually result in death before 10 years of age. Ref.5 Ref.6 Ref.8 Ref.9 Ref.10 Ref.13 Ref.14 Ref.16 Ref.17 Ref.18 Ref.19 Ref.20 Ref.21 Defects in IDUA are the cause of mucopolysaccharidosis type 1H/S (MPS1H/S) [MIM:607015]; also known as Hurler-Scheie syndrome. MPS1H/S is a form of mucopolysaccharidosis type 1, a rare lysosomal storage disease characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. MPS1H/S represents an intermediate phenotype of the MPS1 clinical spectrum. It is characterized by relatively little neurological involvement, but most of the somatic symptoms described for severe MPS1 develop in the early to mid-teens, causing considerable loss of mobility. Defects in IDUA are the cause of mucopolysaccharidosis type 1S (MPS1S) [MIM:607016]; also known as Scheie syndrome. MPS1S is a mild form of mucopolysaccharidosis type 1, a rare lysosomal storage disease characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. Patients with MPS1S may have little or no neurological involvement, normal stature and life span, but present development of joints stiffness, mild hepatosplenomegaly, aortic valve disease and corneal clouding.
Sequence similarities	Belongs to the glycosyl hydrolase 39 family.

Ontologies

Keywords
Cellular component	Lysosome
Coding sequence diversity	Polymorphism
Disease	Disease mutation Mucopolysaccharidosis
Domain	Signal
Molecular function	Glycosidase Hydrolase
PTM	Glycoprotein
Technical term	3D-structure Complete proteome Reference proteome
Gene Ontology (GO)
Biological process	disaccharide metabolic process Traceable author statement. Source: ProtInc
Cellular component	lysosome Inferred from electronic annotation. Source: UniProtKB-SubCell
Molecular function	L-iduronidase activity Traceable author statement. Source: ProtInc cation binding Inferred from electronic annotation. Source: InterPro
Complete GO annotation...

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Signal peptide	1 – 27	27	Potential
	Chain	28 – 653	626	Alpha-L-iduronidase		PRO_0000012200
Sites
	Active site	182	1	Proton donor Potential
	Active site	299	1	Nucleophile Potential
Amino acid modifications
	Glycosylation	110	1	N-linked (GlcNAc...) Ref.4
	Glycosylation	190	1	N-linked (GlcNAc...) Potential
	Glycosylation	336	1	N-linked (GlcNAc...) Potential
	Glycosylation	372	1	N-linked (GlcNAc...) Potential
	Glycosylation	415	1	N-linked (GlcNAc...) Potential
	Glycosylation	451	1	N-linked (GlcNAc...) Potential
Natural variations
	Natural variant	16 – 19	4	Missing in MPS1H.		VAR_003349
	Natural variant	33	1	H → Q. Ref.1 Ref.2 Ref.11 Ref.21 Corresponds to variant rs10794537 [ dbSNP | Ensembl ].		VAR_003350
	Natural variant	51	1	G → D in MPS1H. Ref.10 Ref.21		VAR_003351
	Natural variant	75	1	A → T in MPS1H. Ref.5 Ref.10		VAR_003352
	Natural variant	76	1	Y → C in MPS1S. Ref.21		VAR_066215
	Natural variant	79	1	A → V in MPS1H/S; reduction of activity and protein levels. Ref.20		VAR_020975
	Natural variant	82	1	H → P in MPS1H/S. Ref.6		VAR_003353
	Natural variant	82	1	H → Q Reduction of protein levels. Ref.20		VAR_020976
	Natural variant	84	1	G → R in MPS1H/S. Ref.21		VAR_066216
	Natural variant	89	1	R → Q in MPS1S; in Japanese 21% of alleles. Ref.7 Ref.19		VAR_003354
	Natural variant	89	1	R → W in MPS1S. Ref.14 Ref.21		VAR_003355
	Natural variant	103	1	T → P in MPS1H; uncertain pathological role. Ref.21		VAR_066217
	Natural variant	105	1	R → Q. Ref.20 Ref.21 Corresponds to variant rs3755955 [ dbSNP | Ensembl ].		VAR_003356
	Natural variant	116	1	G → R.		VAR_003357
	Natural variant	133	1	M → I in MPS1H. Ref.19		VAR_020977
	Natural variant	178	1	E → K in MPS1H/S. Ref.21		VAR_066218
	Natural variant	182	1	E → K in MPS1H. Ref.19		VAR_020978
	Natural variant	188	1	F → L in MPS1H/S; associated with R-423. Ref.21		VAR_066219
	Natural variant	208	1	G → D in MPS1H. Ref.19		VAR_020979
	Natural variant	218	1	L → P in MPS1H. Ref.10		VAR_003358
	Natural variant	219	1	G → E in MPS1S. Ref.21		VAR_066220
	Natural variant	238	1	L → Q in MPS1H/S. Ref.20		VAR_020980
	Natural variant	260	1	S → F in MPS1H/S. Ref.19		VAR_020981
	Natural variant	265	1	G → R in MPS1H/S. Ref.21		VAR_066221
	Natural variant	276	1	E → K in MPS1H/S and MPS1S. Ref.21		VAR_066222
	Natural variant	279	1	V → A.		VAR_003359
	Natural variant	300	1	A → T in IDUA pseudodeficiency. Ref.15		VAR_017435
	Natural variant	306	1	W → L in MPS1S. Ref.21		VAR_066223
	Natural variant	315	1	D → Y in MPS1.		VAR_003360
	Natural variant	327	1	A → P in MPS1H and MPS1H/S. Ref.10 Ref.19 Ref.20 Ref.21		VAR_003361
	Natural variant	346	1	L → R in MPS1H/S; 0.4% of normal activity. Ref.18		VAR_017436
	Natural variant	348	1	N → K in MPS1S. Ref.21		VAR_066224
	Natural variant	349 – 350	2	Missing in MPS1H.		VAR_003363
	Natural variant	349	1	D → N in MPS1H.		VAR_003362
	Natural variant	349	1	D → Y in MPS1H. Ref.19		VAR_020982
	Natural variant	350	1	N → I in MPS1S. Ref.19		VAR_020983
	Natural variant	361	1	A → T. Ref.12 Ref.20 Ref.21 Corresponds to variant rs6831280 [ dbSNP | Ensembl ].		VAR_003364
	Natural variant	363	1	R → C in MPS1H/S; loss of activity. Ref.20		VAR_020984
	Natural variant	366	1	T → P in MPS1H. Ref.8		VAR_003365
	Natural variant	380	1	Q → R in MPS1H/S. Ref.19 Ref.20		VAR_003366
	Natural variant	383	1	R → H in MPS1S; 2-3% of normal activity. Ref.14 Ref.19		VAR_003367
	Natural variant	385	1	P → R in MPS1H. Ref.21		VAR_066225
	Natural variant	388	1	T → R in MPS1H. Ref.16		VAR_003368
	Natural variant	396	1	L → LALL in MPS1H.		VAR_003369
	Natural variant	396	1	L → P in MPS1. Ref.21		VAR_066226
	Natural variant	409	1	G → R in MPS1H. Ref.8 Corresponds to variant rs11934801 [ dbSNP | Ensembl ].		VAR_003370
	Natural variant	423	1	S → R in MPS1S and MPS1H/S; associated with L-188 in a patient with MPS1H/S; significant reduction of activity and protein levels. Ref.20 Ref.21		VAR_020985
	Natural variant	436	1	A → P in MPS1H/S. Ref.21		VAR_066227
	Natural variant	445	1	Missing in MPS1S.		VAR_003371
	Natural variant	449	1	H → N. Ref.21		VAR_066228
	Natural variant	454	1	V → I. Ref.20 Ref.21 Corresponds to variant rs73066479 [ dbSNP | Ensembl ].		VAR_003372
	Natural variant	489	1	R → P in MPS1H. Ref.10 Corresponds to variant rs4690226 [ dbSNP | Ensembl ].		VAR_003373
	Natural variant	490	1	L → P in MPS1H/S and MPS1S. Ref.13 Ref.21		VAR_003374
	Natural variant	492	1	R → P in MPS1S. Ref.13 Ref.21		VAR_003375
	Natural variant	496	1	P → L in MPS1H/S. Ref.13		VAR_003376
	Natural variant	496	1	P → R in MPS1H/S. Ref.21		VAR_066229
	Natural variant	504	1	M → T in MPS1H/S. Ref.14		VAR_003377
	Natural variant	533	1	P → R in MPS1H and MPS1H/S; in 3% of the MPS1H patients. Ref.9 Ref.19 Ref.20 Ref.21		VAR_003378
	Natural variant	535	1	L → F in MPS1H/S. Ref.21		VAR_066230
	Natural variant	591	1	A → T. Ref.21		VAR_066231
	Natural variant	602	1	F → I in MPS1H/S; reduction of activity and protein levels. Ref.20		VAR_020986
	Natural variant	619	1	R → G in MPS1H/S; 1.5% of normal activity. Ref.17		VAR_017437
	Natural variant	626	1	W → R in MPS1H/S. Ref.14		VAR_003379
	Natural variant	628	1	R → P in MPS1H/S. Ref.19		VAR_020987
Experimental info
	Sequence conflict	622	1	A → T in AAA51698. Ref.2

Sequences

	Sequence		Length	Mass (Da)
	P35475 [UniParc]. Last modified April 4, 2006. Version 2. Checksum: 9D2399B22FD172BD	FASTA	653	72,670
	10 20 30 40 50 60 MRPLRPRAAL LALLASLLAA PPVAPAEAPH LVHVDAARAL WPLRRFWRST GFCPPLPHSQ 70 80 90 100 110 120 ADQYVLSWDQ QLNLAYVGAV PHRGIKQVRT HWLLELVTTR GSTGRGLSYN FTHLDGYLDL 130 140 150 160 170 180 LRENQLLPGF ELMGSASGHF TDFEDKQQVF EWKDLVSSLA RRYIGRYGLA HVSKWNFETW 190 200 210 220 230 240 NEPDHHDFDN VSMTMQGFLN YYDACSEGLR AASPALRLGG PGDSFHTPPR SPLSWGLLRH 250 260 270 280 290 300 CHDGTNFFTG EAGVRLDYIS LHRKGARSSI SILEQEKVVA QQIRQLFPKF ADTPIYNDEA 310 320 330 340 350 360 DPLVGWSLPQ PWRADVTYAA MVVKVIAQHQ NLLLANTTSA FPYALLSNDN AFLSYHPHPF 370 380 390 400 410 420 AQRTLTARFQ VNNTRPPHVQ LLRKPVLTAM GLLALLDEEQ LWAEVSQAGT VLDSNHTVGV 430 440 450 460 470 480 LASAHRPQGP ADAWRAAVLI YASDDTRAHP NRSVAVTLRL RGVPPGPGLV YVTRYLDNGL 490 500 510 520 530 540 CSPDGEWRRL GRPVFPTAEQ FRRMRAAEDP VAAAPRPLPA GGRLTLRPAL RLPSLLLVHV 550 560 570 580 590 600 CARPEKPPGQ VTRLRALPLT QGQLVLVWSD EHVGSKCLWT YEIQFSQDGK AYTPVSRKPS 610 620 630 640 650 TFNLFVFSPD TGAVSGSYRV RALDYWARPG PFSDPVPYLE VPVPRGPPSP GNP

References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"Human alpha-L-iduronidase: cDNA isolation and expression." Scott H.S., Anson D.S., Orsborn A.M., Nelson P.V., Clements P.R., Morris C.P., Hopwood J.J. Proc. Natl. Acad. Sci. U.S.A. 88:9695-9699(1991) [PubMed: 1946389] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA], VARIANT GLN-33. Tissue: Liver.
[2]	"Structure and sequence of the human alpha-L-iduronidase gene." Scott H.S., Guo X.H., Hopwood J.J., Morris C.P. Genomics 13:1311-1313(1992) [PubMed: 1505961] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA], VARIANT GLN-33.
[3]	"Molecular genetics of mucopolysaccharidosis type I: diagnostic, clinical, and biological implications." Scott H.S., Bunge S., Gal A., Clarke L.A., Morris C.P., Hopwood J.J. Hum. Mutat. 6:288-302(1995) [PubMed: 8680403] [Abstract] Cited for: REVIEW ON VARIANTS.
[4]	"Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry." Chen R., Jiang X., Sun D., Han G., Wang F., Ye M., Wang L., Zou H. J. Proteome Res. 8:651-661(2009) [PubMed: 19159218] [Abstract] Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-110, MASS SPECTROMETRY. Tissue: Liver.
[5]	"Mutation analysis of 19 North American mucopolysaccharidosis type I patients: identification of two additional frequent mutations." Clarke L.A., Nelson P.V., Warrington C.L., Morris C.P., Hopwood J.J., Scott H.S. Hum. Mutat. 3:275-282(1994) [PubMed: 8019563] [Abstract] Cited for: VARIANT MPS1H THR-75.
[6]	"Two novel mutations causing mucopolysaccharidosis type I detected by single strand conformational analysis of the alpha-L-iduronidase gene." Clark L.A., Scott H.S. Hum. Mol. Genet. 2:1311-1312(1993) [PubMed: 8401515] [Abstract] Cited for: VARIANT MPS1H/S PRO-82.
[7]	"Identification of mutations in the alpha-L-iduronidase gene (IDUA) that cause Hurler and Scheie syndromes." Scott H.S., Litjens T., Nelson P.V., Thompson P.R., Brooks D.A., Hopwood J.J., Morris C.P. Am. J. Hum. Genet. 53:973-986(1993) [PubMed: 8213840] [Abstract] Cited for: VARIANT MPS1S GLN-89.
[8]	"Molecular analysis of Hurler syndrome in Druze and Muslim Arab patients in Israel: multiple allelic mutations of the IDUA gene in a small geographic area." Bach G., Moskowitz S.M., Tieu P.T., Matynia A., Neufeld E.F. Am. J. Hum. Genet. 53:330-338(1993) [PubMed: 8328452] [Abstract] Cited for: VARIANTS MPS1H PRO-366 AND ARG-409.
[9]	"Alpha-L-iduronidase mutations (Q70X and P533R) associate with a severe Hurler phenotype." Scott H.S., Litjens T., Nelson P.V., Brooks D.A., Hopwood J.J., Morris C.P. Hum. Mutat. 1:333-339(1992) [PubMed: 1301941] [Abstract] Cited for: VARIANT MPS1H ARG-533.
[10]	"Mucopolysaccharidosis type I: identification of 8 novel mutations and determination of the frequency of the two common alpha-L-iduronidase mutations (W402X and Q70X) among European patients." Bunge S., Kleijer W.J., Steglich C., Beck M., Zuther C., Morris C.P., Schwinger E., Hopwood J.J., Scott H.S., Gal A. Hum. Mol. Genet. 3:861-866(1994) [PubMed: 7951228] [Abstract] Cited for: VARIANTS MPS1H ASP-51; THR-75; PRO-218; PRO-327; PRO-489 AND 16-SER--ALA-19 DEL.
[11]	"PCR detection of two RFLPs in exon I of the alpha-L-iduronidase (IDUA) gene." Scott H.S., Litjens T., Hopwood J.J., Morris C.P. Hum. Genet. 90:327-327(1992) [PubMed: 1362562] [Abstract] Cited for: VARIANT GLN-33.
[12]	"Multiple polymorphisms within the alpha-L-iduronidase gene (IDUA): implications for a role in modification of MPS-I disease phenotype." Scott H.S., Nelson P.V., Litjens T., Hopwood J.J., Morris C.P. Hum. Mol. Genet. 2:1471-1473(1993) [PubMed: 8242073] [Abstract] Cited for: VARIANT THR-361.
[13]	"Four novel mutations underlying mild or intermediate forms of alpha-L-iduronidase deficiency (MPS IS and MPS IH/S)." Tieu P.T., Bach G., Matynia A., Hwang M., Neufeld E.F. Hum. Mutat. 6:55-59(1995) [PubMed: 7550232] [Abstract] Cited for: VARIANTS MPS1H/S PRO-490 AND LEU-496, VARIANT MPS1S PRO-492.
[14]	"Mucopolysaccharidosis type I: identification of 13 novel mutations of the alpha-L-iduronidase gene." Bunge S., Kleijer W.J., Steglich C., Beck M., Schwinger E., Gal A. Hum. Mutat. 6:91-94(1995) [PubMed: 7550242] [Abstract] Cited for: VARIANTS MPS1S TRP-89 AND HIS-383, VARIANT MPS1H 349-ASP-ASN-350 DEL, VARIANTS MPS1H/S THR-504 AND ARG-626.
[15]	"Molecular genetic defect underlying alpha-L-iduronidase pseudodeficiency." Aronovich E.L., Pan D., Whitley C.B. Am. J. Hum. Genet. 58:75-85(1996) [PubMed: 8554071] [Abstract] Cited for: VARIANT IDUA PSEUDODEFICIENCY THR-300.
[16]	"A novel missense mutation in the human IDUA gene associated with a severe Hurler's phenotype." Bartholomew D.W., McClellan J.M. Hum. Mutat. 12:291-291(1998) Cited for: VARIANT MPS1H ARG-388.
[17]	"Mucopolysaccharidosis type I: characterization of novel mutations affecting alpha-L-iduronidase activity." Lee-Chen G.J., Lin S.P., Tang Y.F., Chin Y.W. Clin. Genet. 56:66-70(1999) [PubMed: 10466419] [Abstract] Cited for: VARIANT MPS1H/S GLY-619.
[18]	"Identification and characterization of -3c-g acceptor splice site mutation in human alpha-L-iduronidase associated with mucopolysaccharidosis type IH/S." Teng Y.N., Wang T.R., Hwu W.L., Lin S.P., Lee-Chen G.J. Clin. Genet. 57:131-136(2000) [PubMed: 10735634] [Abstract] Cited for: VARIANT MPS1H/S ARG-346.
[19]	"Identification and characterization of 13 new mutations in mucopolysaccharidosis type I patients." Matte U., Yogalingam G., Brooks D., Leistner S., Schwartz I., Lima L., Norato D.Y., Brum J.M., Beesley C., Winchester B., Giugliani R., Hopwood J.J. Mol. Genet. Metab. 78:37-43(2003) [PubMed: 12559846] [Abstract] Cited for: VARIANTS MPS1H ILE-133; LYS-182; ASP-208; TYR-349 AND ARG-533, VARIANTS MPS1H/S PHE-260; PRO-327; ARG-380 AND PRO-628, VARIANTS MPS1S GLN-89; ILE-350; HIS-383 AND ASP-445 DEL.
[20]	"Identification and molecular characterization of alpha-L-iduronidase mutations present in mucopolysaccharidosis type I patients undergoing enzyme replacement therapy." Yogalingam G., Guo X.H., Muller V.J., Brooks D.A., Clements P.R., Kakkis E.D., Hopwood J.J. Hum. Mutat. 24:199-207(2004) [PubMed: 15300847] [Abstract] Cited for: VARIANTS MPS1H/S VAL-79; GLN-238; PRO-327; CYS-363; ARG-380; ARG-533 AND ILE-602, VARIANT MPS1S ARG-423, VARIANTS GLN-82; GLN-105; THR-361 AND ILE-454, CHARACTERIZATION OF VARIANTS MPS1H/S VAL-79; GLN-238; CYS-363 AND ILE-602, CHARACTERIZATION OF VARIANT MPS1S ARG-423, CHARACTERIZATION OF VARIANT GLN-82.
[21]	"IDUA mutational profiling of a cohort of 102 European patients with mucopolysaccharidosis type I: identification and characterization of 35 novel alpha-L-iduronidase (IDUA) alleles." Bertola F., Filocamo M., Casati G., Mort M., Rosano C., Tylki-Szymanska A., Tuysuz B., Gabrielli O., Grossi S., Scarpa M., Parenti G., Antuzzi D., Dalmau J., Di Rocco M., Vici C.D., Okur I., Rosell J., Rovelli A. , Furlan F., Rigoldi M., Biondi A., Cooper D.N., Parini R. Hum. Mutat. 32:E2189-E2210(2011) [PubMed: 21394825] [Abstract] Cited for: VARIANTS MPS1H/S ARG-84; LYS-178; LEU-188; ARG-265; LYS-276; ARG-423; PRO-436; ARG-496; ARG-533 AND PHE-535, VARIANTS MPS1H ASP-51; PRO-103; PRO-327 AND ARG-385, VARIANTS MPS1S CYS-76; TRP-89; GLU-219; LYS-276; LEU-306; LYS-348; PRO-490 AND PRO-492, VARIANT MPS1 PRO-396, VARIANTS GLN-33; GLN-105; THR-361; ASN-449; ILE-454 AND THR-591.
+	Additional computationally mapped references.

Web resources

GeneReviews

Cross-references

Sequence databases

EMBL
GenBank
DDBJ

M74715 mRNA. Translation: AAA81589.1.
M95740, M95739 Genomic DNA. Translation: AAA51698.1.

IPI

IPI00018879.

PIR

S53645.

RefSeq

NP_000194.2. NM_000203.3.

UniGene

Hs.89560.

3D structure databases

PDBe
RCSB PDB
PDBj

Entry	Method	Resolution (Å)	Chain	Positions	PDBsum
1Y24	model	-	A	36-522	[»]

ProteinModelPortal

P35475.

ModBase

Search...

Protein-protein interaction databases

IntAct

P35475. 1 interaction.

MINT

MINT-1397491.

STRING

P35475.

Protein family/group databases

CAZy

GH39. Glycoside Hydrolase Family 39.

Polymorphism databases

DMDM

92090608.

Proteomic databases

PRIDE

P35475.

Protocols and materials databases

StructuralBiologyKnowledgebase

Search...

Genome annotation databases

Ensembl

ENST00000247933; ENSP00000247933; ENSG00000127415.

GeneID

3425.

KEGG

hsa:3425.

NMPDR

fig|9606.3.peg.3462.

UCSC

uc003gby.1. human.

Organism-specific databases

CTD

3425.

GeneCards

GC04P000970.

H-InvDB

HIX0200643.

HGNC

HGNC:5391. IDUA.

HPA

CAB025901.

MIM

252800. gene.
607014. phenotype.
607015. phenotype.
607016. phenotype.

neXtProt

NX_P35475.

Orphanet

93473. Hurler syndrome.

PharmGKB

PA29638.

GenAtlas

Search...

Phylogenomic databases

eggNOG

prNOG04522.

HOVERGEN

HBG006121.

InParanoid

P35475.

OrthoDB

EOG4THVSS.

PhylomeDB

P35475.

Enzyme and pathway databases

BioCyc

MetaCyc:HS05096-MONOMER.

BRENDA

3.2.1.76. 2681.

Gene expression databases

ArrayExpress

P35475.

Bgee

P35475.

CleanEx

HS_IDUA.

Genevestigator

P35475.

GermOnline

ENSG00000127415. Homo sapiens.

Family and domain databases

InterPro

IPR000514. Glyco_hydro_39.
IPR013781. Glyco_hydro_subgr_catalytic.
IPR017853. Glycoside_hydrolase_SF.
[Graphical view]

Gene3D

G3DSA:3.20.20.80. Glyco_hydro_cat. 1 hit.

KO

K01217.

Pfam

PF01229. Glyco_hydro_39. 1 hit.
[Graphical view]

PRINTS

PR00745. GLHYDRLASE39.

SUPFAM

SSF51445. Glyco_hydro_cat. 1 hit.

PROSITE

PS01027. GLYCOSYL_HYDROL_F39. 1 hit.
[Graphical view]

ProtoNet

Search...

Other

DrugBank

DB00090. Laronidase.

NextBio

13508.

SOURCE

Search...

Entry information

Entry name

IDUA_HUMAN

Accession

Primary (citable) accession number: P35475

Entry history

Integrated into UniProtKB/Swiss-Prot:	June 1, 1994
Last sequence update:	April 4, 2006
Last modified:	December 14, 2011
This is version 110 of the entry and version 2 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation program

Chordata Protein Annotation Program

Disclaimer

Any 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.