KEGG   Homo sapiens (human): 3425Help
Entry
3425              CDS       T01001                                 

Gene name
IDUA, IDA, MPS1
Definition
(RefSeq) iduronidase, alpha-L-
  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
N00610  Dermatan sulfate degradation
N00611  HYAL1 deficiency in dermatan sulfate degradation
N00612  ARSB deficiency in dermatan sulfate degradation
N00613  IDUA deficiency in dermatan sulfate degradation
N00615  Heparan sulfate degradation
N00616  GNS deficiency in heparan sulfate degradation
N00617  GUSB deficiency in heparan sulfate degradation
N00618  NAGLU deficiency in heparan sulfate degradation
N00619  HGSNAT deficiency in heparan sulfate degradation
N00620  SGSH deficiency in heparan sulfate degradation
N00621  IDUA deficiency in heparan sulfate degradation
N10012  Replacement of deficient GALNS
 » show all
Disease
H00128  Mucopolysaccharidosis type I
H00421  Mucopolysaccharidosis
Drug target
Laronidase: D04670
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)
BRITE hierarchy
SSDB OrthologParalogGFIT
Motif
Pfam: Glyco_hydro_39 NHS
Motif
Other DBs
NCBI-GeneID: 3425
NCBI-ProteinID: NP_000194
OMIM: 252800
HGNC: 5391
Ensembl: ENSG00000127415
Vega: OTTHUMG00000088901
Pharos: P35475(Tbio)
UniProt: P35475
Structure
PDB: 

Position
4p16.3
AA seq 653 aa AA seqDB search
MRPLRPRAALLALLASLLAAPPVAPAEAPHLVHVDAARALWPLRRFWRSTGFCPPLPHSQ
ADQYVLSWDQQLNLAYVGAVPHRGIKQVRTHWLLELVTTRGSTGRGLSYNFTHLDGYLDL
LRENQLLPGFELMGSASGHFTDFEDKQQVFEWKDLVSSLARRYIGRYGLAHVSKWNFETW
NEPDHHDFDNVSMTMQGFLNYYDACSEGLRAASPALRLGGPGDSFHTPPRSPLSWGLLRH
CHDGTNFFTGEAGVRLDYISLHRKGARSSISILEQEKVVAQQIRQLFPKFADTPIYNDEA
DPLVGWSLPQPWRADVTYAAMVVKVIAQHQNLLLANTTSAFPYALLSNDNAFLSYHPHPF
AQRTLTARFQVNNTRPPHVQLLRKPVLTAMGLLALLDEEQLWAEVSQAGTVLDSNHTVGV
LASAHRPQGPADAWRAAVLIYASDDTRAHPNRSVAVTLRLRGVPPGPGLVYVTRYLDNGL
CSPDGEWRRLGRPVFPTAEQFRRMRAAEDPVAAAPRPLPAGGRLTLRPALRLPSLLLVHV
CARPEKPPGQVTRLRALPLTQGQLVLVWSDEHVGSKCLWTYEIQFSQDGKAYTPVSRKPS
TFNLFVFSPDTGAVSGSYRVRALDYWARPGPFSDPVPYLEVPVPRGPPSPGNP
NT seq 1962 nt NT seq  +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

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