General Information of Drug-Metabolizing Enzyme (DME) (ID: DEL45C2)

DME Name Iduronate 2-sulfatase (IDS)
Synonyms Idursulfase; Alpha-L-iduronate sulfate sulfatase; Iduronate 2-sulfatase 14 kDa chain; Iduronate 2-sulfatase 42 kDa chain; IDS; SIDS
Gene Name IDS
UniProt ID
IDS_HUMAN
INTEDE ID
DME0108
3D Structure
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2D Sequence (FASTA)
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3D Structure (PDB)
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Gene ID
3423
EC Number EC: 3.1.6.13
Hydrolases
Ester bond hydrolase
Sulfuric ester hydrolase
EC: 3.1.6.13
Lineage Species: Homo sapiens
Kingdom: Metazoa
Phylum: Chordata
Class: Mammalia
Order: Primates
Family: Hominidae
Genus: Homo
Species: Homo sapiens
Sequence
MPPPRTGRGLLWLGLVLSSVCVALGSETQANSTTDALNVLLIIVDDLRPSLGCYGDKLVR
SPNIDQLASHSLLFQNAFAQQAVCAPSRVSFLTGRRPDTTRLYDFNSYWRVHAGNFSTIP
QYFKENGYVTMSVGKVFHPGISSNHTDDSPYSWSFPPYHPSSEKYENTKTCRGPDGELHA
NLLCPVDVLDVPEGTLPDKQSTEQAIQLLEKMKTSASPFFLAVGYHKPHIPFRYPKEFQK
LYPLENITLAPDPEVPDGLPPVAYNPWMDIRQREDVQALNISVPYGPIPVDFQRKIRQSY
FASVSYLDTQVGRLLSALDDLQLANSTIIAFTSDHGWALGEHGEWAKYSNFDVATHVPLI
FYVPGRTASLPEAGEKLFPYLDPFDSASQLMEPGRQSMDLVELVSLFPTLAGLAGLQVPP
RCPVPSFHVELCREGKNLLKHFRFRDLEEDPYLPGNPRELIAYSQYPRPSDIPQWNSDKP
SLKDIKIMGYSIRTIDYRYTVWVGFNPDEFLANFSDIHAGELYFVDSDPLQDHNMYNDSQ
GGDLFQLLMP
Function This enzyme is involved in the degradation pathway of dermatan sulfate and heparan sulfate.
KEGG Pathway
Glycosaminoglycan degradation (hsa00531 )
Lysosome (hsa04142 )
Metabolic pathways (hsa01100 )
Reactome Pathway
HS-GAG degradation (R-HSA-2024096 )
MPS II - Hunter syndrome (R-HSA-2206296 )
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 [6]

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 5.78E-30 3.37E-01 1.36E+00
Alopecia ED70 Skin from scalp 4.00E-03 -1.56E-01 -5.11E-01
Alzheimer's disease 8A20 Entorhinal cortex 1.08E-07 -8.27E-01 -1.05E+00
Ankylosing spondylitis FA92.0 Pheripheral blood 2.83E-01 8.70E-02 5.05E-01
Aortic stenosis BB70 Calcified aortic valve 9.42E-01 5.13E-02 2.73E-01
Apnea 7A40 Hyperplastic tonsil 5.45E-01 -8.28E-04 -1.12E-02
Arthropathy FA00-FA5Z Peripheral blood 8.38E-02 1.65E-01 6.99E-01
Asthma CA23 Nasal and bronchial airway 2.00E-05 -3.59E-01 -8.07E-01
Atopic dermatitis EA80 Skin 9.68E-06 2.42E-01 1.87E+00
Autism 6A02 Whole blood 6.46E-01 1.78E-02 6.11E-02
Autoimmune uveitis 9A96 Peripheral monocyte 2.60E-05 -3.07E-01 -1.68E+00
Autosomal dominant monocytopenia 4B04 Whole blood 7.42E-05 -8.45E-01 -2.45E+00
Bacterial infection of gingival 1C1H Gingival tissue 3.28E-07 -2.08E-01 -8.16E-01
Batten disease 5C56.1 Whole blood 8.68E-01 -4.74E-03 -2.79E-02
Behcet's disease 4A62 Peripheral blood 9.95E-01 -3.16E-02 -1.35E-01
Bipolar disorder 6A60-6A6Z Prefrontal cortex 4.21E-01 -8.48E-02 -2.02E-01
Bladder cancer 2C94 Bladder tissue 3.78E-03 -3.15E-01 -1.45E+00
Breast cancer 2C60-2C6Z Breast tissue 1.68E-11 1.44E-01 5.13E-01
Cardioembolic stroke 8B11.20 Whole blood 8.92E-01 -8.99E-02 -3.22E-01
Cervical cancer 2C77 Cervical tissue 3.47E-01 -7.25E-02 -3.43E-01
Childhood onset rheumatic disease FA20.Z Peripheral blood 5.35E-01 -1.75E-02 -1.56E-02
Chronic hepatitis C 1E51.1 Whole blood 3.26E-01 -7.34E-02 -2.91E-01
Chronic obstructive pulmonary disease CA22 Lung tissue 5.91E-01 -8.20E-03 -2.98E-02
Chronic obstructive pulmonary disease CA22 Small airway epithelium 1.18E-03 2.30E-01 5.22E-01
Chronic rhinosinusitis CA0A Sinus mucosa tissue 7.76E-02 -2.89E-01 -1.16E+00
Colon cancer 2B90 Colon tissue 2.03E-15 -1.73E-01 -7.57E-01
Coronary artery disease BA80-BA8Z Peripheral blood 2.98E-01 3.92E-01 9.81E-01
Diffuse large B-cell lymphoma 2A81 Tonsil tissue 9.55E-01 -6.09E-03 -1.44E-02
Endometriosis GA10 Endometrium tissue 1.60E-02 2.63E-01 5.62E-01
Familial hypercholesterolemia 5C80.00 Peripheral blood 8.65E-01 -6.77E-03 -3.11E-02
Familial hypercholesterolemia 5C80.00 Whole blood 1.49E-04 -4.08E-01 -1.39E+00
Gastric cancer 2B72 Gastric tissue 5.29E-01 2.97E-01 7.37E-01
Glioblastopma 2A00.00 Nervous tissue 2.14E-91 -1.26E+00 -1.20E+00
Glioma 2A00.0Y-2A00.0Z Brain stem tissue 6.11E-01 -4.62E-04 -3.93E-03
Glioma 2A00.0Y-2A00.0Z White matter tissue 9.92E-01 2.26E-01 5.06E-01
Head and neck cancer 2D42 Head and neck tissue 5.48E-11 -3.44E-01 -5.70E-01
HIV-associated neurocognitive impairment 8A2Y-8A2Z White matter tissue 4.94E-01 2.34E-02 3.69E-02
Huntington's disease 8A01.10 Whole blood 9.47E-01 -1.02E-01 -4.29E-01
Idiopathic pulmonary fibrosis CB03.4 Lung tissue 7.30E-01 -9.44E-02 -3.93E-01
Immunodeficiency 4A00-4A20 Peripheral blood 1.01E-02 -1.13E-01 -1.16E+00
Influenza 1E30 Whole blood 8.77E-01 -7.48E-02 -1.14E-01
Interstitial cystitis GC00.3 Bladder tissue 4.03E-01 7.57E-02 9.09E-01
Intracranial aneurysm 8B01.0 Intracranial artery 1.16E-01 -1.47E-01 -5.70E-01
Irritable bowel syndrome DD91.0 Rectal colon tissue 5.80E-01 -5.10E-02 -1.86E-01
Ischemic stroke 8B11 Peripheral blood 5.89E-01 6.82E-02 1.98E-01
Juvenile idiopathic arthritis FA24 Peripheral blood 1.68E-01 -1.27E-01 -4.35E-01
Lateral sclerosis 8B60.4 Skin 2.94E-01 -3.45E-01 -8.23E-01
Lateral sclerosis 8B60.4 Cervical spinal cord 6.38E-02 3.49E-01 1.31E+00
Liver cancer 2C12.0 Liver tissue 7.88E-03 1.49E-01 6.40E-01
Liver failure DB99.7-DB99.8 Liver tissue 6.87E-01 -3.20E-02 -1.81E-01
Lung cancer 2C25 Lung tissue 1.47E-10 -1.54E-01 -5.83E-01
Lupus erythematosus 4A40 Whole blood 2.60E-01 -1.36E-01 -2.75E-01
Major depressive disorder 6A70-6A7Z Hippocampus 9.20E-01 -1.33E-02 -3.38E-02
Major depressive disorder 6A70-6A7Z Whole blood 9.43E-01 5.74E-03 1.36E-02
Melanoma 2C30 Skin 9.46E-01 1.40E-02 2.26E-02
Multiple myeloma 2A83.1 Peripheral blood 5.70E-01 4.59E-02 1.61E-01
Multiple myeloma 2A83.1 Bone marrow 4.44E-05 5.28E-01 2.59E+00
Multiple sclerosis 8A40 Plasmacytoid dendritic cells 4.07E-01 1.37E-01 4.41E-01
Myelodysplastic syndrome 2A36-2A3Z Bone marrow 9.97E-03 -2.96E-01 -6.15E-01
Myelofibrosis 2A20.2 Whole blood 7.21E-04 -2.20E-01 -1.22E+00
Myocardial infarction BA41-BA50 Peripheral blood 8.14E-03 3.43E-01 6.90E-01
Myopathy 8C70.6 Muscle tissue 7.11E-01 4.94E-02 1.81E-01
Neonatal sepsis KA60 Whole blood 1.43E-01 4.15E-02 9.46E-02
Neuroectodermal tumour 2A00.11 Brain stem tissue 2.20E-03 -3.05E-01 -1.05E+00
Non-alcoholic fatty liver disease DB92 Liver tissue 7.27E-01 -2.00E-02 -1.09E-01
Obesity related type 2 diabetes 5A11 Omental adipose tissue 2.60E-01 8.79E-02 5.45E-01
Olive pollen allergy CA08.00 Peripheral blood 1.53E-01 -2.15E-01 -6.78E-01
Oral cancer 2B6E Oral tissue 3.48E-03 1.62E-01 6.37E-01
Osteoarthritis FA00-FA0Z Synovial tissue 5.38E-01 1.44E-01 3.33E-01
Osteoporosis FB83.1 Bone marrow 1.76E-01 1.11E-01 1.05E+00
Ovarian cancer 2C73 Ovarian tissue 2.73E-02 -5.04E-01 -1.14E+00
Pancreatic cancer 2C10 Pancreas 6.28E-05 3.17E-01 1.37E+00
Parkinson's disease 8A00.0 Substantia nigra tissue 6.35E-02 -1.57E-01 -1.61E+00
Pediatric respiratory syncytial virus infection CA40.11 Peripheral blood 6.45E-01 -3.40E-02 -1.66E-01
Pituitary cancer 2D12 Pituitary tissue 1.22E-01 -3.65E-02 -1.18E-01
Pituitary gonadotrope tumour 2D12 Pituitary tissue 5.20E-01 -1.35E-01 -3.80E-01
Polycystic ovary syndrome 5A80.1 Vastus lateralis muscle 7.23E-01 3.01E-02 3.47E-01
Polycythemia vera 2A20.4 Whole blood 5.06E-03 -1.24E-01 -6.54E-01
Pompe disease 5C51.3 Biceps muscle 9.12E-01 1.47E-02 5.19E-02
Preterm birth KA21.4Z Myometrium 5.82E-01 9.50E-02 2.87E-01
Prostate cancer 2C82 Prostate 1.80E-02 2.77E-01 9.61E-01
Psoriasis EA90 Skin 5.95E-01 -3.17E-03 -1.25E-02
Rectal cancer 2B92 Rectal colon tissue 1.14E-01 1.49E-01 1.09E+00
Renal cancer 2C90-2C91 Kidney 1.16E-04 3.98E-01 1.81E+00
Retinoblastoma 2D02.2 Uvea 1.66E-02 2.56E-01 1.76E+00
Rheumatoid arthritis FA20 Synovial tissue 1.05E-03 6.23E-01 2.06E+00
Rhinovirus infection CA42.1 Nasal epithelium tissue 1.97E-01 -9.47E-02 -4.56E-01
Schizophrenia 6A20 Prefrontal cortex 7.84E-03 -1.83E-01 -3.36E-01
Schizophrenia 6A20 Superior temporal cortex 2.54E-01 1.05E-01 4.46E-01
Scleroderma 4A42.Z Whole blood 3.14E-01 4.89E-02 2.71E-01
Seizure 8A60-8A6Z Whole blood 9.90E-01 -1.02E-03 -3.16E-03
Sensitive skin EK0Z Skin 1.91E-01 4.60E-02 2.79E-01
Sepsis with septic shock 1G41 Whole blood 2.97E-04 2.19E-01 4.65E-01
Shwachman-Diamond syndrome 3A70.0 Bone marrow 8.85E-01 -1.02E-01 -4.38E-01
Sickle cell disease 3A51.0-3A51.3 Peripheral blood 1.30E-02 1.75E-01 9.36E-01
Simpson golabi behmel syndrome LD2C Adipose tissue 8.84E-01 9.77E-02 4.40E-01
Sjogren's syndrome 4A43.2 Salivary gland tissue 1.24E-05 -3.33E-01 -1.01E+01
Skin cancer 2C30-2C3Z Skin 5.79E-01 -5.30E-02 -1.59E-01
Thrombocythemia 3B63 Whole blood 5.05E-03 -9.72E-02 -5.14E-01
Thrombocytopenia 3B64 Whole blood 6.01E-01 3.46E-01 5.50E-01
Thyroid cancer 2D10 Thyroid 6.42E-11 1.95E-01 9.41E-01
Tibial muscular dystrophy 8C75 Muscle tissue 2.78E-02 -1.74E-01 -1.02E+00
Tuberous sclerosis complex LD2D.2 Perituberal tissue 2.62E-03 -1.46E+00 -3.18E+00
Type 2 diabetes 5A11 Liver tissue 4.25E-01 -4.42E-03 -2.70E-02
Ureter cancer 2C92 Urothelium 5.05E-01 -8.99E-02 -3.24E-01
Uterine cancer 2C78 Endometrium tissue 2.28E-01 -2.68E-02 -9.66E-02
Vitiligo ED63.0 Skin 1.63E-01 2.19E-01 1.09E+00
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⏷ Show the Full List of DME Expression Under 107 Diseases

The Drug Therapeutic Target (DTT) Role of This DME

DME DTT Name Iduronate 2-sulfatase (IDS) DTT Info
DME DTT Type Successful
1 Approved Drug(s) Targeting This DTT
Drug Name Drug ID Indication ICD 11 Highest Status REF
Idursulfase DM30SXV Hunter syndrome 5C56.31 Approved [1]
4 Clinical Trial Drug(s) Targeting This DTT
Drug Name Drug ID Indication ICD 11 Highest Status REF
Recombinant human iduronate-2-sulfatase DMOWQAF Hunter syndrome 5C56.31 Phase 3 [2]
DNL310 DMO3QAZ Mucopolysaccharidosis II 5C56.31 Phase 2/3 [3]
RGX-121 DMU3PD7 Mucopolysaccharidosis 5C56.3 Phase 1/2 [4]
SB-913 DM98YFK Mucopolysaccharidosis 5C56.3 Phase 1/2 [5]

References

1 2006 drug approvals: finding the niche. Nat Rev Drug Discov. 2007 Feb;6(2):99-101.
2 The effect of recombinant human iduronate-2-sulfatase (Idursulfase) on growth in young patients with mucopolysaccharidosis type II. PLoS One. 2014 Jan 13;9(1):e85074.
3 Clinical pipeline report, company report or official report of Denali
4 Clinical pipeline report, company report or official report of REGENXBIO.
5 ClinicalTrials.gov (NCT03041324) Ascending Dose Study of Genome Editing by the Zinc Finger Nuclease (ZFN) Therapeutic SB-913 in Subjects With MPS II. U.S. National Institutes of Health.
6 MFN human ref: Chondroitin sulfate degradation.