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 Maximum [Da] | Comment | Organism |
---|---|---|---|
76000 | - |
recombinant IDS, immunoblot analysis | Homo sapiens |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
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 | - |
fibroblast | control | Homo sapiens | - |
hepatocyte | IDS enzyme activity in mucopolysaccharidosis type II patient at 10 months post-cord blood stem cell transplantation | Homo sapiens | - |
Kupffer cell | IDS enzyme activity in mucopolysaccharidosis type II patient at 10 months post-cord blood stem cell transplantation | Homo sapiens | - |
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 | - |
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 | - |
Synonyms | Comment | Organism |
---|---|---|
I2S | - |
Homo sapiens |
iduronate-2-sulfatase | - |
Homo sapiens |
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 |