General Information of Drug-Metabolizing Enzyme (DME) (ID: DELTYX6)

DME Name Alpha-L-iduronidase (IDUA)
Synonyms Iduronidase; L-iduronidase; Alpha iduronidase; Laronidase; IDUA
Gene Name IDUA
UniProt ID
IDUA_HUMAN
INTEDE ID
DME0104
3D Structure
Download
2D Sequence (FASTA)
Download
3D Structure (PDB)
Download
Gene ID
3425
EC Number EC: 3.2.1.76
Hydrolases
Glycosylase
O/S-glycosyl compound glycosidase
EC: 3.2.1.76
Lineage Species: Homo sapiens
Kingdom: Metazoa
Phylum: Chordata
Class: Mammalia
Order: Primates
Family: Hominidae
Genus: Homo
Species: Homo sapiens
Sequence
MRPLRPRAALLALLASLLAAPPVAPAEAPHLVHVDAARALWPLRRFWRSTGFCPPLPHSQ
ADQYVLSWDQQLNLAYVGAVPHRGIKQVRTHWLLELVTTRGSTGRGLSYNFTHLDGYLDL
LRENQLLPGFELMGSASGHFTDFEDKQQVFEWKDLVSSLARRYIGRYGLAHVSKWNFETW
NEPDHHDFDNVSMTMQGFLNYYDACSEGLRAASPALRLGGPGDSFHTPPRSPLSWGLLRH
CHDGTNFFTGEAGVRLDYISLHRKGARSSISILEQEKVVAQQIRQLFPKFADTPIYNDEA
DPLVGWSLPQPWRADVTYAAMVVKVIAQHQNLLLANTTSAFPYALLSNDNAFLSYHPHPF
AQRTLTARFQVNNTRPPHVQLLRKPVLTAMGLLALLDEEQLWAEVSQAGTVLDSNHTVGV
LASAHRPQGPADAWRAAVLIYASDDTRAHPNRSVAVTLRLRGVPPGPGLVYVTRYLDNGL
CSPDGEWRRLGRPVFPTAEQFRRMRAAEDPVAAAPRPLPAGGRLTLRPALRLPSLLLVHV
CARPEKPPGQVTRLRALPLTQGQLVLVWSDEHVGSKCLWTYEIQFSQDGKAYTPVSRKPS
TFNLFVFSPDTGAVSGSYRVRALDYWARPGPFSDPVPYLEVPVPRGPPSPGNP
Function This enzyme catalyses the hydrolysis of unsulfated alpha-L-iduronosidic linkages in dermatan sulfate.
KEGG Pathway
Glycosaminoglycan degradation (hsa00531 )
Lysosome (hsa04142 )
Metabolic pathways (hsa01100 )
Reactome Pathway
HS-GAG degradation (R-HSA-2024096 )
MPS I - Hurler syndrome (R-HSA-2206302 )
CS/DS degradation (R-HSA-2024101 )

Molecular Interaction Atlas (MIA) of This DME

Molecular Interaction Atlas (MIA) Jump to Detail Molecular Interaction Atlas of This DME
1 Approved Drug(s) Metabolized by This DME
Drug Name Drug ID Indication ICD 11 Highest Status REF
Chondroitin sulfate DM0N19Y N. A. N. A. Phase 4 [3]

Molecular Expression Atlas (MEA) of This DME

Molecular Expression Atlas (MEA) Jump to Detail Molecular Expression Atlas of This DME
Disease Name ICD 11 Studied Tissue p-value Fold-Change Z-score
Acute myelocytic leukaemia 2B33.1 Bone marrow 8.34E-04 9.34E-02 3.53E-01
Alopecia ED70 Skin from scalp 3.43E-03 1.75E-01 2.93E-01
Alzheimer's disease 8A20 Entorhinal cortex 8.93E-02 -3.78E-02 -2.39E-01
Ankylosing spondylitis FA92.0 Pheripheral blood 4.57E-01 3.31E-03 1.34E-02
Aortic stenosis BB70 Calcified aortic valve 5.53E-01 2.72E-01 6.57E-01
Apnea 7A40 Hyperplastic tonsil 7.18E-01 6.97E-02 6.43E-01
Arthropathy FA00-FA5Z Peripheral blood 9.76E-01 -5.23E-02 -3.28E-01
Asthma CA23 Nasal and bronchial airway 6.18E-04 2.92E-01 5.71E-01
Atopic dermatitis EA80 Skin 2.57E-02 -9.13E-02 -1.07E+00
Autism 6A02 Whole blood 9.07E-01 6.34E-02 2.42E-01
Autoimmune uveitis 9A96 Peripheral monocyte 4.74E-02 -2.26E-01 -2.67E+00
Autosomal dominant monocytopenia 4B04 Whole blood 3.08E-01 -4.94E-02 -3.91E-01
Bacterial infection of gingival 1C1H Gingival tissue 2.43E-04 1.56E-01 5.39E-01
Batten disease 5C56.1 Whole blood 2.75E-01 1.73E-01 1.54E+00
Behcet's disease 4A62 Peripheral blood 3.64E-01 3.63E-02 1.86E-01
Bipolar disorder 6A60-6A6Z Prefrontal cortex 8.26E-01 -3.40E-02 -1.81E-01
Bladder cancer 2C94 Bladder tissue 2.39E-02 1.95E-01 8.95E-01
Breast cancer 2C60-2C6Z Breast tissue 5.71E-03 7.04E-02 2.61E-01
Cardioembolic stroke 8B11.20 Whole blood 6.21E-03 -1.89E-01 -6.95E-01
Cervical cancer 2C77 Cervical tissue 5.16E-01 -7.75E-02 -3.08E-01
Childhood onset rheumatic disease FA20.Z Peripheral blood 1.14E-01 1.12E-01 5.74E-01
Chronic hepatitis C 1E51.1 Whole blood 1.73E-01 4.00E-02 3.16E-01
Chronic obstructive pulmonary disease CA22 Lung tissue 3.22E-01 -7.88E-02 -3.23E-01
Chronic obstructive pulmonary disease CA22 Small airway epithelium 6.46E-01 1.43E-02 5.57E-02
Chronic rhinosinusitis CA0A Sinus mucosa tissue 5.19E-01 -2.16E-02 -5.52E-01
Colon cancer 2B90 Colon tissue 2.83E-07 -1.16E-01 -4.75E-01
Coronary artery disease BA80-BA8Z Peripheral blood 4.80E-01 -8.23E-02 -2.83E-01
Diffuse large B-cell lymphoma 2A81 Tonsil tissue 8.30E-01 2.81E-02 1.53E-01
Endometriosis GA10 Endometrium tissue 6.66E-01 8.06E-02 3.00E-01
Familial hypercholesterolemia 5C80.00 Peripheral blood 8.98E-02 1.25E-01 7.23E-01
Familial hypercholesterolemia 5C80.00 Whole blood 9.68E-03 2.37E-01 1.32E+00
Gastric cancer 2B72 Gastric tissue 3.35E-01 -2.06E-01 -5.59E-01
Glioblastopma 2A00.00 Nervous tissue 8.75E-01 -3.44E-03 -1.09E-02
Glioma 2A00.0Y-2A00.0Z Brain stem tissue 5.14E-01 -2.67E-01 -6.74E-01
Glioma 2A00.0Y-2A00.0Z White matter tissue 6.27E-01 5.56E-02 1.77E-01
Head and neck cancer 2D42 Head and neck tissue 3.30E-02 -4.12E-02 -2.04E-01
HIV-associated neurocognitive impairment 8A2Y-8A2Z White matter tissue 3.05E-02 1.75E-01 6.96E-01
Huntington's disease 8A01.10 Whole blood 2.12E-01 1.73E-01 7.19E-01
Idiopathic pulmonary fibrosis CB03.4 Lung tissue 5.29E-01 5.18E-02 2.58E-01
Immunodeficiency 4A00-4A20 Peripheral blood 1.28E-01 6.07E-02 4.63E-01
Influenza 1E30 Whole blood 1.26E-01 4.67E-01 1.74E+00
Interstitial cystitis GC00.3 Bladder tissue 5.27E-03 3.44E-01 2.19E+00
Intracranial aneurysm 8B01.0 Intracranial artery 8.65E-01 3.36E-02 1.42E-01
Irritable bowel syndrome DD91.0 Rectal colon tissue 2.40E-01 -3.33E-02 -1.47E-01
Ischemic stroke 8B11 Peripheral blood 4.29E-02 1.00E-01 1.18E+00
Juvenile idiopathic arthritis FA24 Peripheral blood 1.30E-03 -4.37E-01 -7.23E-01
Lateral sclerosis 8B60.4 Skin 4.29E-01 -1.58E-01 -7.82E-01
Lateral sclerosis 8B60.4 Cervical spinal cord 3.29E-02 -4.13E-01 -1.23E+00
Liver cancer 2C12.0 Liver tissue 5.77E-01 1.51E-02 4.56E-02
Liver failure DB99.7-DB99.8 Liver tissue 1.81E-01 9.94E-02 3.55E-01
Lung cancer 2C25 Lung tissue 8.42E-05 -4.67E-02 -2.26E-01
Lupus erythematosus 4A40 Whole blood 4.47E-01 2.52E-03 8.63E-03
Major depressive disorder 6A70-6A7Z Hippocampus 1.14E-01 -8.00E-02 -5.37E-01
Major depressive disorder 6A70-6A7Z Whole blood 8.87E-01 1.45E-02 6.03E-02
Melanoma 2C30 Skin 2.29E-01 -1.84E-01 -2.65E-01
Multiple myeloma 2A83.1 Peripheral blood 9.75E-01 1.27E-02 1.12E-01
Multiple myeloma 2A83.1 Bone marrow 7.30E-02 1.60E-01 7.01E-01
Multiple sclerosis 8A40 Plasmacytoid dendritic cells 8.09E-01 1.01E-01 3.79E-01
Myelodysplastic syndrome 2A36-2A3Z Bone marrow 3.53E-01 -8.70E-03 -4.19E-02
Myelofibrosis 2A20.2 Whole blood 7.73E-02 -1.46E-01 -1.27E+00
Myocardial infarction BA41-BA50 Peripheral blood 2.43E-01 -6.90E-03 -1.14E-02
Myopathy 8C70.6 Muscle tissue 1.44E-01 1.16E-01 8.24E-01
Neonatal sepsis KA60 Whole blood 9.86E-07 -2.37E-01 -8.88E-01
Neuroectodermal tumour 2A00.11 Brain stem tissue 1.28E-01 -1.33E-01 -6.01E-01
Non-alcoholic fatty liver disease DB92 Liver tissue 6.08E-01 9.90E-02 2.73E-01
Obesity related type 2 diabetes 5A11 Omental adipose tissue 7.13E-02 1.72E-01 1.13E+00
Olive pollen allergy CA08.00 Peripheral blood 6.51E-01 2.40E-02 1.30E-01
Oral cancer 2B6E Oral tissue 2.02E-04 -3.03E-01 -9.16E-01
Osteoarthritis FA00-FA0Z Synovial tissue 2.91E-01 4.05E-01 5.73E-01
Osteoporosis FB83.1 Bone marrow 1.74E-03 3.78E-01 4.03E+00
Ovarian cancer 2C73 Ovarian tissue 6.55E-02 -1.38E-01 -5.17E-01
Pancreatic cancer 2C10 Pancreas 7.81E-03 -3.07E-01 -8.08E-01
Parkinson's disease 8A00.0 Substantia nigra tissue 7.42E-01 8.83E-02 6.41E-01
Pediatric respiratory syncytial virus infection CA40.11 Peripheral blood 1.32E-01 -1.64E-01 -1.06E+00
Pituitary cancer 2D12 Pituitary tissue 2.49E-02 2.39E-01 1.00E+00
Pituitary gonadotrope tumour 2D12 Pituitary tissue 3.01E-01 1.35E-01 5.11E-01
Polycystic ovary syndrome 5A80.1 Vastus lateralis muscle 5.72E-01 2.40E-02 1.18E-01
Polycythemia vera 2A20.4 Whole blood 3.50E-05 -6.83E-02 -6.81E-01
Pompe disease 5C51.3 Biceps muscle 5.58E-01 -9.96E-02 -5.53E-01
Preterm birth KA21.4Z Myometrium 5.58E-01 -1.06E-01 -6.66E-01
Prostate cancer 2C82 Prostate 1.59E-02 3.73E-01 5.76E-01
Psoriasis EA90 Skin 1.81E-04 1.86E-01 4.11E-01
Rectal cancer 2B92 Rectal colon tissue 2.75E-02 -1.99E-01 -1.03E+00
Renal cancer 2C90-2C91 Kidney 9.82E-01 -5.39E-02 -1.65E-01
Retinoblastoma 2D02.2 Uvea 9.70E-01 3.92E-02 1.92E-01
Rheumatoid arthritis FA20 Synovial tissue 4.29E-03 4.46E-01 1.23E+00
Rhinovirus infection CA42.1 Nasal epithelium tissue 5.12E-01 -6.42E-02 -4.04E-01
Schizophrenia 6A20 Prefrontal cortex 6.41E-01 -2.58E-04 -6.67E-04
Schizophrenia 6A20 Superior temporal cortex 2.03E-01 -1.10E-02 -7.47E-02
Scleroderma 4A42.Z Whole blood 3.73E-02 2.25E-01 1.21E+00
Seizure 8A60-8A6Z Whole blood 5.69E-01 -4.57E-02 -2.49E-01
Sensitive skin EK0Z Skin 4.50E-01 -5.15E-02 -6.34E-01
Sepsis with septic shock 1G41 Whole blood 2.74E-20 -2.12E-01 -8.21E-01
Shwachman-Diamond syndrome 3A70.0 Bone marrow 2.19E-01 -2.13E-01 -1.00E+00
Sickle cell disease 3A51.0-3A51.3 Peripheral blood 9.30E-01 1.43E-01 5.95E-01
Simpson golabi behmel syndrome LD2C Adipose tissue 8.18E-01 -3.13E-02 -1.91E-01
Sjogren's syndrome 4A43.2 Salivary gland tissue 8.36E-01 -4.12E-02 -2.21E-01
Skin cancer 2C30-2C3Z Skin 4.57E-04 -1.99E-01 -5.09E-01
Thrombocythemia 3B63 Whole blood 2.17E-02 -8.18E-02 -7.99E-01
Thrombocytopenia 3B64 Whole blood 2.79E-01 9.82E-01 8.51E-01
Thyroid cancer 2D10 Thyroid 1.43E-01 2.58E-02 1.13E-01
Tibial muscular dystrophy 8C75 Muscle tissue 1.15E-01 -1.89E-01 -7.23E-01
Tuberous sclerosis complex LD2D.2 Perituberal tissue 2.57E-01 1.34E-01 1.22E+00
Type 2 diabetes 5A11 Liver tissue 7.03E-02 -3.25E-01 -1.29E+00
Ureter cancer 2C92 Urothelium 5.26E-01 3.06E-03 1.09E-02
Uterine cancer 2C78 Endometrium tissue 3.41E-11 -1.83E-01 -4.78E-01
Vitiligo ED63.0 Skin 7.62E-01 6.27E-02 2.18E-01
------------------------------------------------------------------------------------
⏷ Show the Full List of DME Expression Under 107 Diseases

The Drug Therapeutic Target (DTT) Role of This DME

DME DTT Name Alpha-L-iduronidase (IDUA) DTT Info
DME DTT Type Clinical trial
2 Clinical Trial Drug(s) Targeting This DTT
Drug Name Drug ID Indication ICD 11 Highest Status REF
SB-318 DMA8Z4N Mucopolysaccharidosis 5C56.3 Phase 1/2 [1]
RGX-111 DMU87HW Mucopolysaccharidosis 5C56.3 Phase 1 [2]

References

1 ClinicalTrials.gov (NCT02702115) Ascending Dose Study of Genome Editing by the Zinc Finger Nuclease (ZFN) Therapeutic SB-318 in Subjects With MPS I. U.S. National Institutes of Health.
2 Clinical pipeline report, company report or official report of REGENXBIO.
3 MFN human ref: Chondroitin sulfate degradation.