Iduronate Sulfatase Protein is an important component in the neurobiology of neurodegenerative diseases. This page provides detailed information about its structure, function, and role in disease processes.
Iduronate sulfatase (IDS) is a lysosomal sulfatase encoded by the IDS gene. It catalyzes the removal of sulfate groups from iduronic acid residues in glycosaminoglycans (heparan sulfate and dermatan sulfate). Deficiency causes Mucopolysaccharidosis type II (Hunter syndrome), an X-linked lysosomal storage disorder. [1]
| Property | Value | [2]
|----------|-------| [3]
| Protein Name | Iduronate sulfatase | [4]
| Gene | IDS |
| UniProt ID | P22304 |
| Molecular Weight | 56 kDa (precursor) |
| Subcellular Localization | Lysosome |
| Protein Family | Sulfatase family |
Iduronate sulfatase:
The enzyme removes sulfate groups from:
This desulfation is essential for complete lysosomal degradation of GAGs.
Accumulation of partially degraded GAGs causes:
[5] Scarcy M, et al. (2005). Iduronate-2-sulfatase gene mutations in patients with Hunter syndrome. Human Mutation.
[1:1] Muenzer J, et al. (2009). Idursulfase for the treatment of Hunter syndrome. Genetics in Medicine.
The study of Iduronate Sulfatase Protein has evolved significantly over the past decades. Research in this area has revealed important insights into the underlying mechanisms of neurodegeneration and continues to drive therapeutic development.
Historical context and key discoveries in this field have shaped our current understanding and will continue to guide future research directions.
Walkley SU, et al. "Lysosomal storage diseases: Pathways and therapeutic strategies." Nat Rev Neurol. Nat Rev Neurol. 2023. ↩︎ ↩︎
Parenti G, et al. "Lysosomal storage diseases: From pathophysiology to therapy." Adv Pharmacol. Adv Pharmacol. 2023. ↩︎
Sun A. "Lysosomal storage disease overview." J Biochem. J Biochem. 2022. ↩︎
Wang RY, et al. "Enzyme replacement therapy for mucopolysaccharidoses." Mol Genet Metab. Mol Genet Metab. 2021. ↩︎
Platt FM, et al. "Lysosomal storage disorders." Nat Rev Dis Primers. Nat Rev Dis Primers. 2024. ↩︎