Lysosomin sulfataaseista haku.

Lysosomal sulfatases: a growing family.

Lübke T, Damme M. Biochem J. 2020 Oct 30;477(20):3963-3983. doi: 10.1042/BCJ20200586. PMID: 33120425 Review.

The majority of the so far described sulfatases localize intracellularly to lysosomes, where they act in different catabolic pathways. Mutations in genes coding for lysosomal sulfatases lead to an accumulation of the sulfated substrates in lysosomes …

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Brain delivery and activity of a lysosomal enzyme using a blood-brain barrier transport vehicle in mice.

Ullman JC, Arguello A, Getz JA, Bhalla A, Mahon CS, Wang J, Giese T, Bedard C, Kim DJ, Blumenfeld JR, Liang N, Ravi R, Nugent AA, Davis SS, Ha C, Duque J, Tran HL, Wells RC, Lianoglou S, Daryani VM, Kwan W, Solanoy H, Nguyen H, Earr T, Dugas JC, Tuck MD, Harvey JL, Reyzer ML, Caprioli RM, Hall S, Poda S, Sanchez PE, Dennis MS, Gunasekaran K, Srivastava A, Sandmann T, Henne KR, Thorne RG, Di Paolo G, Astarita G, Diaz D, Silverman AP, Watts RJ, Sweeney ZK, Kariolis MS, Henry AG. Sci Transl Med. 2020 May 27;12(545):eaay1163. doi: 10.1126/scitranslmed.aay1163. PMID: 32461331

Most lysosomal storage diseases (LSDs) involve progressive central nervous system (CNS) impairment, resulting from deficiency of a lysosomal enzyme. ...We demonstrate that ETV fusions containing iduronate 2-sulfatase (ETV:IDS), the lysosomal enzyme def …

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Molecular architecture determines brain delivery of a transferrin receptor-targeted lysosomal enzyme.

Arguello A, Mahon CS, Calvert MEK, Chan D, Dugas JC, Pizzo ME, Thomsen ER, Chau R, Damo LA, Duque J, Fang M, Giese T, Kim DJ, Liang N, Nguyen HN, Solanoy H, Tsogtbaatar B, Ullman JC, Wang J, Dennis MS, Diaz D, Gunasekaran K, Henne KR, Lewcock JW, Sanchez PE, Troyer MD, Harris JM, Scearce-Levie K, Shan L, Watts RJ, Thorne RG, Henry AG, Kariolis MS. J Exp Med. 2022 Mar 7;219(3):e20211057. doi: 10.1084/jem.20211057. Epub 2022 Feb 28. PMID: 35226042 Free PMC article.

Here, we characterized the pharmacological behavior of two distinct TfR-targeted platforms fused to iduronate 2-sulfatase (IDS), a lysosomal enzyme deficient in mucopolysaccharidosis type II (MPS II), and compared the relative brain exposures and functional activiti …

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Mammalian Sulfatases: Biochemistry, Disease Manifestation, and Therapy.

Mashima R, Nakanishi M. Int J Mol Sci. 2022 Jul 24;23(15):8153. doi: 10.3390/ijms23158153. PMID: 35897729 Free PMC article. Review.

They are commonly activated by the unusual amino acid formylglycine, which is formed from cysteine at the catalytic center, mediated by a formylglycine-generating enzyme as a post-translational modification. Sulfatases are expressed in various cellular compartments such as …

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Sulfatases and human disease.

Diez-Roux G, Ballabio A. Annu Rev Genomics Hum Genet. 2005;6:355-79. doi: 10.1146/annurev.genom.6.080604.162334. PMID: 16124866 Review.

The importance of sulfatases in human metabolism is underscored by the presence of at least eight human monogenic diseases caused by the deficiency of individual sulfatases. Sulfatase activity requires a unique posttranslational modification, which is impaire …

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Sulfatases: structure, mechanism, biological activity, inhibition, and synthetic utility.

Hanson SR, Best MD, Wong CH. Angew Chem Int Ed Engl. 2004 Nov 5;43(43):5736-63. doi: 10.1002/anie.200300632. PMID: 15493058 Review.

These findings have increased interest in sulfatases and in targeting them for therapeutic endeavors. Although numerous sulfatases have been identified, the wide scope of their biological activity is only beginning to emerge. Herein, accounts of the diversity and gr …

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"Pseudodeficiencies" of lysosomal hydrolases.

Thomas GH. Am J Hum Genet. 1994 Jun;54(6):934-40. PMID: 7911004 Free PMC article. Review. No abstract available.

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A novel protein modification generating an aldehyde group in sulfatases: its role in catalysis and disease.

von Figura K, Schmidt B, Selmer T, Dierks T. Bioessays. 1998 Jun;20(6):505-10. doi: 10.1002/(SICI)1521-1878(199806)20:6<505::AID-BIES9>3.0.CO;2-K. PMID: 9699462 Review.

In multiple sulfatase deficiency, a rare human lysosomal storage disorder, all known sulfatases are synthesized as catalytically poorly active polypeptides. ...Analysis of one of these prokaryotic sulfatases, however, revealed the presence of the C alp …

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Arylsulfatase K, a novel lysosomal sulfatase.

Wiegmann EM, Westendorf E, Kalus I, Pringle TH, Lübke T, Dierks T. J Biol Chem. 2013 Oct 18;288(42):30019-30028. doi: 10.1074/jbc.M113.499541. Epub 2013 Aug 28. PMID: 23986440 Free PMC article.

A novel member of this family, arylsulfatase K (ARSK), was identified bioinformatically through its conserved sulfatase signature sequence directing posttranslational generation of the catalytic formylglycine residue in sulfatases. ...ARSK mRNA expression was found …

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Lysosomal degradation of heparin and heparan sulphate.

Freeman C, Hopwood J. Adv Exp Med Biol. 1992;313:121-34. doi: 10.1007/978-1-4899-2444-5_13. PMID: 1442257 Review. No abstract available.