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Idua Gene Detail
Summary
  • Symbol
    Idua
  • Name
    iduronidase, alpha-L
  • Feature Type
    protein coding gene
  • IDs
    MGI:96418
    NCBI Gene: 15932
  • Alliance
    genome feature page
  • Transcription Start Sites
    4 TSS
Location &
Maps
more
  • Sequence Map
    Chr5:108808197-108832423 bp, + strand
    From Ensembl annotation of GRCm39
  • View this region in JBrowse
  • Genome Browsers
    Ensembl | UCSC | NCBI
  • Genetic Map
    Chromosome 5, 53.24 cM
  • Mapping Data
    6 experiments
Strain
Comparison
more
  • SNPs within 2kb
    583 from dbSNP Build 142
  • Strain Annotations
    19
  • RFLP
    6
For selected strains:
Strain Gene Model ID Feature Type Coordinates Select Strains
C57BL/6J MGI_C57BL6J_96418
 protein coding gene Chr5:108808197-108833312 (+)
129S1/SvImJ ENSMUSG00200021894
 protein coding gene Chr5:102557706-102581587 (+)
A/J ENSMUSG00195030880
 protein coding gene Chr5:102014573-102038605 (+)
AKR/J ENSMUSG00220050572
 protein coding gene Chr5:101565039-101589617 (+)
BALB/cJ ENSMUSG00180040880
 protein coding gene Chr5:102167400-102191432 (+)
C3H/HeJ ENSMUSG00175035031
 protein coding gene Chr5:104215867-104239892 (+)
C57BL/6NJ ENSMUSG00215024019
 protein coding gene Chr5:102211182-102235409 (+)
CAROLI/EiJ MGP_CAROLIEiJ_G0027526
 protein coding gene Chr5:101542104-101566099 (+)
CAST/EiJ ENSTCUG00005021264
 protein coding gene Chr5:101449912-101474223 (+)
CBA/J ENSMUSG00210039234
 protein coding gene Chr5:102973452-102997479 (+)
DBA/2J ENSMUSG00185045703
 protein coding gene Chr5:105871968-105896202 (+)
FVB/NJ ENSMUSG00205035551
 protein coding gene Chr5:101900094-101924111 (+)
JF1/MsJ ENSUMUG00000013208
 protein coding gene Chr5:108929579-108953306 (+)
LP/J ENSMUSG00230029454
 protein coding gene Chr5:110098734-110122620 (+)
NOD/ShiLtJ ENSMUSG00190027779
 protein coding gene Chr5:101866951-101890979 (+)
NZO/HlLtJ ENSMUSG00225025294
 protein coding gene Chr5:117720603-117744488 (+)
PWK/PhJ ENSLUMG00010037060
 protein coding gene Chr5:100916191-100939965 (+)
SPRET/EiJ ENSMSPG00010045121
 protein coding gene Chr5:104037877-104054275 (+)
WSB/EiJ ENSIUOG00005039466
 protein coding gene Chr5:104594809-104619365 (+)



Homology
more
  • Human Ortholog
    IDUA, alpha-L-iduronidase
  • Vertebrate Orthologs
    3
Vertebrate Orthology Source
Alliance of Genome Resources
  • Human Ortholog
    IDUA, alpha-L-iduronidase
  • Synonyms
    IDA, MPS1, MPSI
  • Links
    NCBI Gene ID: 3425
    UniProt: P35475

  • Chr Location
    4p16.3; chr4:986997-1008344 (+)  GRCh38
Human Diseases
more
  • Diseases
    2 with Idua mouse models; 4 with human IDUA associations

Human Disease Mouse Models
      
IDs
 View 5 models
      
IDs
 View 1 model
      
IDs
IDs
IDs
Click on a disease name to see all genes associated with that disease.

  • Mutations/Alleles
    3 with disease annotations
  • References
    9 with disease annotations
Mutations,
Alleles, and
Phenotypes
less
  • Phenotype Summary
    130 phenotypes from 6 alleles in 8 genetic backgrounds
    18 phenotypes from multigenic genotypes
    76 phenotype references
Phenotype Overview

adipose tissue
behavior/neurological
cardiovascular system
cellular
craniofacial
digestive/alimentary system
embryo
endocrine/exocrine glands
growth/size/body
hearing/vestibular/ear
hematopoietic system
homeostasis/metabolism
integument
immune system
limbs/digits/tail
liver/biliary system
mortality/aging
muscle
nervous system
pigmentation
renal/urinary system
reproductive system
respiratory system
skeleton
taste/olfaction
neoplasm
vision/eye

Click cells to view annotations.
Targeted mutants show lysosomal storage in multiple tissues, increased urinary GAG, craniofacial and skeletal defects, increased body weight, impaired habituation and long-term memory for aversive training, reduced ventricular function with valve insufficiency, and progressive hearing loss.
Gene Ontology
(GO)
Classifications
less
  • All GO Annotations
    61
  • GO References
    21
Molecular Function

carbohydrate derivative binding
cytoskeletal protein binding
DNA binding
enzyme regulator
hydrolase
ligase
lipid binding
oxidoreductase
RNA binding
signaling receptor activity
signaling receptor binding
transcription
transferase
transporter
Biological Process

carbohydrate derivative metabolism
cell differentiation
cell population proliferation
cellular component organization
DNA-templated transcription
establishment of localization
homeostatic process
immune system process
lipid metabolic process
programmed cell death
protein metabolic process
response to stimulus
signaling
system development
Cellular Component

cell projection
cytoplasmic vesicle
cytoskeleton
cytosol
endoplasmic reticulum
endosome
extracellular region
Golgi apparatus
mitochondrion
membraneless organelle
nucleus
organelle envelope
organelle lumen
plasma membrane
protein-containing complex
synapse
vacuole
Click cells to view annotations.
Expression
less
Expression Overview

early conceptus
embryo ectoderm
embryo endoderm
embryo mesoderm
embryo mesenchyme
extraembryonic component
alimentary system
auditory system
branchial arches
cardiovascular system
connective tissue
endocrine system
exocrine system
hemolymphoid system
integumental system
limbs
liver and biliary system
musculoskeletal system
nervous system
olfactory system
reproductive system
respiratory system
urinary system
visual system
Click cells to view annotations.


  • Assay Results
    691
  • Tissues
    94
  • cDNA Data
    9
  • Literature Summary
    1
  • Comparison Matrix
    Gene Expression + Phenotype
    • Sequences &
    Gene Models
    less
    Representative SequencesLengthStrain/SpeciesFlank
    genomic ENSMUSG00000033540 Ensembl Gene Model | MGI Sequence Detail 24227 C57BL/6J ±  kb
    transcript ENSMUST00000112563 Ensembl | MGI Sequence Detail 3373 Not Applicable  
    polypeptide ENSMUSP00000108182 Ensembl | MGI Sequence Detail 643 Not Applicable  
    For the selected sequence
    Protein
    Information
    less
    Molecular
    Reagents
    less
    • All nucleic 12
      Genomic 3
      cDNA 9

      Microarray probesets 5
    Other
    Accession IDs
    less
    MGD-MRK-11188
    References
    more
    • Summaries
      All 106
      Developmental Gene Expression 1
      Diseases 9
      Gene Ontology 21
      Phenotypes 76
    • Earliest
      J:134667 Roderick TH, et al., Two radiation-induced chromosomal inversions in mice (Mus musculus). Proc Natl Acad Sci U S A. 1970 Oct;67(2):961-7
    • Latest
      J:386083 Hurt SC, et al., Antibodies to recombinant human alpha-L-iduronidase prevent disease correction in cortical bone in MPS I mice. Mol Ther Methods Clin Dev. 2025 Mar 13;33(1):101405
    Contributing Projects:
    Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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    Funding Information
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    Send questions and comments to User Support.     		last database update
    09/08/2026
    MGI 6.24     		The Jackson Laboratory