0000000001165771

AUTHOR

Tadao Orii

showing 2 related works from this author

Heparan sulfate levels in mucopolysaccharidoses and mucolipidoses.

2004

Glycosaminoglycans are accumulated in both mucopolysaccharidoses (MPS) and mucolipidoses (ML). MPS I, II, III and VII and ML II and ML III patients cannot properly degrade heparan sulphate (HS). In spite of the importance of HS storage in the metabolic pathway in these diseases, blood and urine HS levels have not been determined systematically using a simple and economical method. Using a new ELISA method using anti-HS antibodies, HS concentrations in blood and urine were determined in MPS and ML II and ML III patients. HS concentrations were determined in 156 plasma samples from MPS I (n = 23), MPS II (n = 26), MPS III (n = 24), MPS IV (n = 62), MPS VI (n = 5), MPS VII (n = 5), ML II (n = …

congenital hereditary and neonatal diseases and abnormalitiesAdolescentMucopolysaccharidosisEnzyme-Linked Immunosorbent AssayUrineSignificant elevationGlycosaminoglycanchemistry.chemical_compoundMucolipidosesGeneticsmedicineHumansElisa methodskin and connective tissue diseasesChildGenetics (clinical)Chromatography High Pressure LiquidGlycosaminoglycansDose-Response Relationship DrugChemistryHeparinInfant Newbornnutritional and metabolic diseasesMucolipidosesInfantHeparan sulfateMucopolysaccharidosesmedicine.diseaseMolecular biologyDose–response relationshipBiochemistryChemistry ClinicalChild PreschoolHeparitin SulfateBiomarkersJournal of inherited metabolic disease
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Development and testing of new screening method for keratan sulfate in mucopolysaccharidosis IVA.

2004

Mucopolysaccharidosis IVA (MPS IVA), a progressive lysosomal storage disease, causes skeletal dysplasia through excessive storage of keratan sulfate (KS). We developed an ELISA-sandwich assay that used a MAb specific to KS. Forty-five blood and 59 urine specimens from MPS IVA patients (ages 1–65 y) were analyzed to determine whether KS concentration is a suitable marker for early diagnosis and longitudinal assessment of disease severity. Blood specimens were obtained from patients categorized as phenotypically severe (n = 36) and milder (n = 9). Urine specimens were also analyzed from patients categorized as severe (n = 56) and milder (n = 12), respectively. Blood KS levels (101–1525 ng/mL)…

Adultmedicine.medical_specialtyPathologyAdolescentMucopolysaccharidosisStatistics as TopicEnzyme-Linked Immunosorbent AssayUrineGastroenterologyMucopolysaccharidosis Type IVAExcretionDiagnosis Differentialchemistry.chemical_compoundInternal medicinemedicineLysosomal storage diseaseHumansGenetic TestingChildAgedGlycosaminoglycansCreatininebusiness.industryInfantMucopolysaccharidosis IVReproducibility of ResultsMiddle Agedmedicine.diseasechemistryDysplasiaKeratan SulfateChild PreschoolPediatrics Perinatology and Child HealthMucopolysaccharidosis IVsense organsbusinessBiomarkersPediatric research
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