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Literature summary for 3.1.6.13 extracted from

  • Araya, K.; Sakai, N.; Mohri, I.; Kagitani-Shimono, K.; Okinaga, T.; Hashii, Y.; Ohta, H.; Nakamichi, I.; Aozasa, K.; Taniike, M.; Ozono, K.
    Localized donor cells in brain of a Hunter disease patient after cord blood stem cell transplantation (2009), Mol. Genet. Metab., 98, 255-263.
    View publication on PubMed
Search for more literature for 3.1.6.13

Application

Application Comment Organism
medicine efficacy of cord blood stem cell transplantation for Hunter disease (deficiency of IDS) is judged to be insufficient for the brain at 10 months post-therapy, but the pathological detection of donor-derived cells in the brain parenchyma suggests the potential of hematopoietic stem cell transplantation for treatment of neurological symptoms in Hunter disease Homo sapiens

Molecular Weight [Da]

Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
76000
-
 recombinant IDS, immunoblot analysis Homo sapiens

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
brain IDS is present in many cells of the mucopolysaccharidosis type II post-cord blood stem cell transplantation patient as well as in the non-mucopolysaccharidosis type II control. Many IDS-immunoreactive large cells are found in the non-mucopolysaccharidosis type II control, whereas only a few IDS-immunoreactive small cells are found in the mucopolysaccharidosis type II post-cord blood stem cell transplantation patient. Most IDS-positive small cells are localized in perivascular spaces in the mucopolysaccharidosis type II post-cord blood stem cell transplantation patient. Many of them are in the distended Virchow-Robin spaces, and some of them in the brain parenchyma. No IDS-immunoreactive neurons in the mucopolysaccharidosis type II post-cord blood stem cell transplantation patient. IDS is present exclusively in CD68-positive microglia/monocytes in the brain of Hunter disease patient, whereas that in the normal brain is also detected in neurons and oligodendrocytes Homo sapiens
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fibroblast control Homo sapiens
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hepatocyte IDS enzyme activity in mucopolysaccharidosis type II patient at 10 months post-cord blood stem cell transplantation Homo sapiens
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Kupffer cell IDS enzyme activity in mucopolysaccharidosis type II patient at 10 months post-cord blood stem cell transplantation Homo sapiens
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liver IDS enzyme activity in mucopolysaccharidosis type II patient at 10 months post-cord blood stem cell transplantation reaches about 40% of the normal control level Homo sapiens
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additional information in cerebrum, very low IDS enzyme activity in mucopolysaccharidosis type II patient at 10 months post-cord blood stem cell transplantation, IDS enzyme activity reaches only 1% of the normal control level Homo sapiens
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Synonyms

Synonyms Comment Organism
I2S
-
 Homo sapiens
iduronate-2-sulfatase
-
 Homo sapiens

General Information

General Information Comment Organism
physiological function a 6-year-old male suffering from a severe type of Hunter disease (deficiency of IDS) with cord blood stem cell transplantation died at 10 months post-therapy due to a laryngeal post-transplantation lymphoproliferative disorder Homo sapiens