Idua Gene Summary [Mouse]
Enables L-iduronidase activity. Involved in heparan sulfate proteoglycan catabolic process. Acts upstream of or within several processes, including learning or memory; proteoglycan catabolic process; and skeletal system development. Located in extracellular space. Is active in lysosomal lumen. Used to study mucopolysaccharidosis I and otitis media. Human ortholog(s) of this gene implicated in Scheie syndrome; mucopolysaccharidosis I; mucopolysaccharidosis Ih; and mucopolysaccharidosis Ih/s. Orthologous to human IDUA (alpha-L-iduronidase). [provided by Alliance of Genome Resources, Jul 2025]
Details
| Type | Protein Coding |
| Official Symbol | Idua |
| Official Name | iduronidase, alpha-L [Source:MGI Symbol;Acc:MGI:96418] |
| Ensembl ID | ENSMUSG00000033540 |
| Bio databases IDs | |
| Aliases | iduronidase, alpha-L |
| Synonyms | 6030426D08, alpha-L-iduronidase, IDA, IDURONIDASE alpha-L-, iduronidase, alpha-L, IDURONIDASE α-L-, iduronidase, α-L, MPS1, MPSI, α-L-iduronidase |
| Species | Mouse, Mus musculus |
| Orthologies |
Protein Domains
A protein domain is a distinct structural or functional region within a protein that can evolve, function, and exist independently of the rest of the protein chain. These domains in mouse Idua often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- L-iduronidase
- Fibronectin-III type domain
- enzyme
- receptor binding
- Glycosyl hydrolases
Top Findings
The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
regulates
- glycosaminoglycan
- chondroitin sulfate B
- sulfate
- heparan sulfate proteoglycan
- heparan sulfate
- disaccharide
- enzyme
- ganglioside GM3
- heparin
- hexosaminidase
disease
- non-insulin-dependent diabetes mellitus
- schizophrenia
- Alzheimer disease
- mucopolysaccharidosis type I
- mucopolysaccharidosis, type IH/S
- Hurler disease
- mucopolysaccharidosis, mps-I-s
- hearing loss
- calcium oxalate nephrolithiasis
- calcium oxalate nephrolithiasis type 1
phenotypes
- Purkinje cell degeneration
- abnormal Purkinje cell morphology
- abnormal QRS complex
- abnormal aorta elastic fiber morphology
- abnormal aorta smooth muscle morphology
- abnormal aortic valve morphology
- abnormal autopod morphology
- abnormal avoidance learning behavior
- abnormal axial skeleton morphology
- abnormal basicranium morphology
- abnormal cardiac muscle relaxation
- abnormal cerebellar cortex morphology
- abnormal cervical vertebrae morphology
- abnormal chondrocyte morphology
- abnormal circadian behavior
- abnormal circadian regulation of heart rate
- abnormal circadian temperature homeostasis
- abnormal circulating enzyme level
- abnormal craniofacial morphology
- abnormal digit morphology
- abnormal endocardium morphology
- abnormal external auditory canal morphology
- abnormal eye morphology
- abnormal foam cell morphology
- abnormal foramen magnum morphology
- abnormal gait
- abnormal heart electrocardiography waveform feature
- abnormal heart left ventricle outflow tract morphology
- abnormal heart ventricle morphology
- abnormal incisor morphology
- abnormal inner ear morphology
- abnormal learning/memory/conditioning
- abnormal limb morphology
- abnormal long bone diaphysis morphology
- abnormal long bone hypertrophic chondrocyte zone
- abnormal long bone morphology
- abnormal long term spatial reference memory
- abnormal lysosome morphology
- abnormal mandibular condyloid process morphology
- abnormal metabolism
- abnormal microglial cell morphology
- abnormal microglial cell physiology
- abnormal mitral valve morphology
- abnormal myocardial fiber morphology
- abnormal myocardium layer morphology
- abnormal neuron morphology
- abnormal response to novel object
- abnormal rib morphology
- abnormal spatial learning
- abnormal tibia morphology
- abnormal tricuspid valve morphology
- abnormal vertebrae morphology
- abnormal zygomatic arch morphology
- abnormal zygomatic bone morphology
- aortic valve regurgitation
- broad face
- broad snout
- cochlear ganglion degeneration
- cochlear hair cell degeneration
- congestive heart failure
- deafness
- decreased body temperature
- decreased cardiac output
- decreased heart rate variability
- decreased percent body fat/body weight
- decreased startle reflex
- decreased survivor rate
- decreased ventricle muscle contractility
- decreased vertical activity
- disheveled coat
- enlarged heart
- hip dislocation
- hypoactivity
- increased anxiety-related response
- increased body weight
- increased bone mineral density
- increased collagen deposition in the muscles
- increased cranium width
- increased diameter of femur
- increased left ventricle weight
- increased or absent threshold for auditory brainstem response
- increased osteoblast cell number
- increased susceptibility to otitis media
- increased systemic arterial systolic blood pressure
- increased urine glycosaminoglycan level
- kyphosis
- lysosomal protein accumulation
- mitral valve regurgitation
- ocular hypertelorism
- organ of Corti degeneration
- pale liver
- postnatal growth retardation
- premature death
- prolonged P wave
- prolonged QRS complex duration
- prominent nasal bridge
- sensorineural hearing loss
- short femur
- short snout
- sparse hair
- spiral ligament degeneration
- thick interventricular septum
- thick ventricular wall
role in cell
- function
- degeneration
- abnormal morphology
- number
- organization
- morphogenesis
Subcellular Expression
Locations within the cell where the protein is known or predicted to be active, providing insight into its function and cellular context.
- Cytoplasm
- Extracellular Space
- lysosome
- lysosomal compartment
Gene Ontology Annotations
Describes the biological processes, cellular components, and molecular functions associated with the mouse Idua gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- dermatan sulfate catabolic process
- heparan sulfate proteoglycan catabolic process
- heparin catabolic process
- disaccharide metabolic process
- glycosaminoglycan catabolic process
Cellular Component
Where in the cell the gene product is active
- extracellular vesicular exosome
- lysosomal lumen
Molecular Function
What the gene product does at the molecular level
- L-iduronidase activity
- receptor binding