KEGG   Homo sapiens (human): 3425
Entry
3425              CDS       T01001                                 
Symbol
IDUA, IDA, MPS1, MPSI
Name
(RefSeq) alpha-L-iduronidase
  KO
K01217  L-iduronidase [EC:3.2.1.76]
Organism
hsa  Homo sapiens (human)
Pathway
hsa00531  Glycosaminoglycan degradation
hsa01100  Metabolic pathways
hsa04142  Lysosome
Module
hsa_M00076  Dermatan sulfate degradation
hsa_M00078  Heparan sulfate degradation
Network
nt06012  Glycosaminoglycan degradation
  Element
N00610  Dermatan sulfate degradation
N00615  Heparan sulfate degradation
N10012  Replacement of deficient IDUA
Disease
H00128  Mucopolysaccharidosis type I
H00421  Mucopolysaccharidosis
Drug target
Laronidase: D04670<JP/US>
Brite
KEGG Orthology (KO) [BR:hsa00001]
 09100 Metabolism
  09107 Glycan biosynthesis and metabolism
   00531 Glycosaminoglycan degradation
    3425 (IDUA)
 09140 Cellular Processes
  09141 Transport and catabolism
   04142 Lysosome
    3425 (IDUA)
Enzymes [BR:hsa01000]
 3. Hydrolases
  3.2  Glycosylases
   3.2.1  Glycosidases, i.e. enzymes that hydrolyse O- and S-glycosyl compounds
    3.2.1.76  L-iduronidase
     3425 (IDUA)
SSDB
Motif
Pfam: Glyco_hydro_39 NHS
Other DBs
NCBI-GeneID: 3425
NCBI-ProteinID: NP_000194
OMIM: 252800
HGNC: 5391
Ensembl: ENSG00000127415
Pharos: P35475(Tbio)
UniProt: P35475
Structure
Position
4:986997..1008351
AA seq 653 aa
MRPLRPRAALLALLASLLAAPPVAPAEAPHLVHVDAARALWPLRRFWRSTGFCPPLPHSQ
ADQYVLSWDQQLNLAYVGAVPHRGIKQVRTHWLLELVTTRGSTGRGLSYNFTHLDGYLDL
LRENQLLPGFELMGSASGHFTDFEDKQQVFEWKDLVSSLARRYIGRYGLAHVSKWNFETW
NEPDHHDFDNVSMTMQGFLNYYDACSEGLRAASPALRLGGPGDSFHTPPRSPLSWGLLRH
CHDGTNFFTGEAGVRLDYISLHRKGARSSISILEQEKVVAQQIRQLFPKFADTPIYNDEA
DPLVGWSLPQPWRADVTYAAMVVKVIAQHQNLLLANTTSAFPYALLSNDNAFLSYHPHPF
AQRTLTARFQVNNTRPPHVQLLRKPVLTAMGLLALLDEEQLWAEVSQAGTVLDSNHTVGV
LASAHRPQGPADAWRAAVLIYASDDTRAHPNRSVAVTLRLRGVPPGPGLVYVTRYLDNGL
CSPDGEWRRLGRPVFPTAEQFRRMRAAEDPVAAAPRPLPAGGRLTLRPALRLPSLLLVHV
CARPEKPPGQVTRLRALPLTQGQLVLVWSDEHVGSKCLWTYEIQFSQDGKAYTPVSRKPS
TFNLFVFSPDTGAVSGSYRVRALDYWARPGPFSDPVPYLEVPVPRGPPSPGNP
NT seq 1962 nt   +upstreamnt  +downstreamnt
atgcgtcccctgcgcccccgcgccgcgctgctggcgctcctggcctcgctcctggccgcg
cccccggtggccccggccgaggccccgcacctggtgcatgtggacgcggcccgcgcgctg
tggcccctgcggcgcttctggaggagcacaggcttctgccccccgctgccacacagccag
gctgaccagtacgtcctcagctgggaccagcagctcaacctcgcctatgtgggcgccgtc
cctcaccgcggcatcaagcaggtccggacccactggctgctggagcttgtcaccaccagg
gggtccactggacggggcctgagctacaacttcacccacctggacgggtacctggacctt
ctcagggagaaccagctcctcccagggtttgagctgatgggcagcgcctcgggccacttc
actgactttgaggacaagcagcaggtgtttgagtggaaggacttggtctccagcctggcc
aggagatacatcggtaggtacggactggcgcatgtttccaagtggaacttcgagacgtgg
aatgagccagaccaccacgactttgacaacgtctccatgaccatgcaaggcttcctgaac
tactacgatgcctgctcggagggtctgcgcgccgccagccccgccctgcggctgggaggc
cccggcgactccttccacaccccaccgcgatccccgctgagctggggcctcctgcgccac
tgccacgacggtaccaacttcttcactggggaggcgggcgtgcggctggactacatctcc
ctccacaggaagggtgcgcgcagctccatctccatcctggagcaggagaaggtcgtcgcg
cagcagatccggcagctcttccccaagttcgcggacacccccatttacaacgacgaggcg
gacccgctggtgggctggtccctgccacagccgtggagggcggacgtgacctacgcggcc
atggtggtgaaggtcatcgcgcagcatcagaacctgctactggccaacaccacctccgcc
ttcccctacgcgctcctgagcaacgacaatgccttcctgagctaccacccgcaccccttc
gcgcagcgcacgctcaccgcgcgcttccaggtcaacaacacccgcccgccgcacgtgcag
ctgttgcgcaagccggtgctcacggccatggggctgctggcgctgctggatgaggagcag
ctctgggccgaagtgtcgcaggccgggaccgtcctggacagcaaccacacggtgggcgtc
ctggccagcgcccaccgcccccagggcccggccgacgcctggcgcgccgcggtgctgatc
tacgcgagcgacgacacccgcgcccaccccaaccgcagcgtcgcggtgaccctgcggctg
cgcggggtgccccccggcccgggcctggtctacgtcacgcgctacctggacaacgggctc
tgcagccccgacggcgagtggcggcgcctgggccggcccgtcttccccacggcagagcag
ttccggcgcatgcgcgcggctgaggacccggtggccgcggcgccccgccccttacccgcc
ggcggccgcctgaccctgcgccccgcgctgcggctgccgtcgcttttgctggtgcacgtg
tgtgcgcgccccgagaagccgcccgggcaggtcacgcggctccgcgccctgcccctgacc
caagggcagctggttctggtctggtcggatgaacacgtgggctccaagtgcctgtggaca
tacgagatccagttctctcaggacggtaaggcgtacaccccggtcagcaggaagccatcg
accttcaacctctttgtgttcagcccagacacaggtgctgtctctggctcctaccgagtt
cgagccctggactactgggcccgaccaggccccttctcggaccctgtgccgtacctggag
gtccctgtgccaagagggcccccatccccgggcaatccatga

DBGET integrated database retrieval system