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Fluorometric measurement of urinary alpha-L-iduronidase activity.

Fluorometric measurement of urinary alpha-L-iduronidase activity. Research Abstract Details 

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  • Fluorometric measurement of urinary alpha-L-iduronidase activity. Abstract Text:

    m isemuraM Isemura,h kosakaH Kosaka,t ikenakaT Ikenaka,r kidoR Kido,t yoshimuraT Yoshimura,

    A fluorogenic substrate for alpha-L-iduronidase, 4-methylumbelliferyl alpha-L-iduronide, has been newly synthesized and the enzyme activity has been measured in urine samples obtained from normal persons and patients suffering from mucopolysaccharidosis. Urine samples derived from a patient with Scheie syndrome showed greatly reduced activity compared with a normal adult at a similar age. This patient exhibited a high level of urinary excretion of dermatan sulfate and heparan sulfate, which could be interpreted in terms of her low alpha-L-iduronidase activity. The use of the fluorogenic substrate has some advantages over existing methods because of the high sensitivity and the relative ease of handling, and it should be useful not only for diagnosis but also for following the purification process of the enzyme.

    Fluorometric measurement of urinary alpha-L-iduronidase activity. Publishing Authors By Initials

    m isemuraM Isemura,h kosakaH Kosaka,t ikenakaT Ikenaka,r kidoR Kido,t yoshimuraT Yoshimura,

    For similar congenital, hereditary, and neonatal diseases and abnormalities: genetic diseases, inborn: metabolism, inborn errors: carbohydrate metabolism, inborn errors: mucopolysaccharidoses: mucopolysaccharidosis i research abstracts see: congenital, hereditary, and neonatal diseases and abnormalities: genetic diseases, inborn: metabolism, inborn errors: carbohydrate metabolism, inborn errors: mucopolysaccharidoses: mucopolysaccharidosis i research

    PUBMED ID PMID:

    MEDLINE DATE:

    Fluorometric measurement of urinary alpha-L-iduronidase activity. Journal Published:

    PUBLICATION TYPE: Journal Article

    Journal: Journal of biochemistry

    VOLUME: 84

    Page Numbers: 627-32

    Journal Abbreviation: J. Biochem.

    ISSN: 0021-924X

    DAY: 19

    MONTH: Sep

    YEAR: 1978

    Fluorometric measurement of urinary alpha-L-iduronidase activity. Information

    Number of References:

    LANGUAGE: eng

    NlmUniqueID: 376600

    Fluorometric measurement of urinary alpha-L-iduronidase activity. Keywords Mesh Terms:

    KEYWORDS: Mucopolysaccharidosis I

    MESH TERMS: urine

    Chemical & Substance for Abstract: Fluorometric measurement of urinary alpha-L-iduronidase activity. Information

    Substance Name: Iduronidase

    Registry Number: EC 3.2.1.76

    Grant and Affiliation Information for Fluorometric measurement of urinary alpha-L-iduronidase activity.

    AFFILIATION:

    Country: JAPAN

    JAPAN Research PublicationJAPAN Research Publication

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    MEDLINETA: J Biochem

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