Literature DB >> 406106

A radioactive substrate and assay for alpha-L-iduronidase.

P Di Natale, I G Leder, E F Neufeld.   

Abstract

A novel radioactive substrate for alpha-L-iduronidase, anhydro[3H]mannitol-iduronide has been prepared. This compound is hydrolysed by alpha-L-iduronidase to yield anhydro[3H]mannitol, which can be separated from the substrate and counted. The assay, described for use with fibroblast homogenates, is satisfactory for the diagnosis of mucopolysaccharidosis I.

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Year:  1977        PMID： 406106     DOI： 10.1016/0009-8981(77)90224-8

Source DB:  PubMed          Journal:  Clin Chim Acta        ISSN： 0009-8981            Impact factor:   3.786


  2 in total

1.  Biochemical diagnosis of mucopolysaccharidoses: experience of 297 diagnoses in a 15-year period (1977-1991).

Authors:  P Di Natale; T Annella; A Daniele; T De Luca; E Morabito; R Pallini; P Rosario; G Spagnuolo
Journal:  J Inherit Metab Dis       Date:  1993       Impact factor: 4.982

2.  Human alpha-L-iduronidase. Catalytic properties and an integrated role in the lysosomal degradation of heparan sulphate.

Authors:  C Freeman; J J Hopwood
Journal:  Biochem J       Date:  1992-03-15       Impact factor: 3.857
  2 in total

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