Literature DB >> 6421718

Properties of alpha-L-iduronidase in cultured skin fibroblasts from alpha-L-iduronidase-deficient patients.

S Fujibayashi, R Minami, Y Ishikawa, K Wagatsuma, T Nakao, S Tsugawa.   

Abstract

On DEAE cellulose column chromatography, alpha-L-iduronidase in cultured skin fibroblasts was resolved into two distinct components, forms A and B. They had similar Km values for 4-methylumbelliferyl-alpha-L-iduronide, but differed in pH optima and thermal stability. Form B was more heat-stable than form A. Residual alpha-L-iduronidase activity in Hurler fibroblasts was heat-stable, while that in Scheie fibroblasts was heat-labile, and moreover, that in Hurler-Scheie compound fibroblasts lay intermediate between Hurler and Scheie syndromes. These findings demonstrated that Hurler syndrome, Scheie syndrome and Hurler-Scheie compound were enzymatically distinguishable.

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Year:  1984        PMID： 6421718     DOI： 10.1007/bf00286515

Source DB:  PubMed          Journal:  Hum Genet        ISSN： 0340-6717            Impact factor:   4.132


  11 in total

1.  The relationship of alpha-L-iduronidase and Hurler corrective factor.

Authors:  L J Shapiro; C W Hall; I G Leder; E F Neufeld
Journal:  Arch Biochem Biophys       Date:  1976-01       Impact factor: 4.013

2.  Protein measurement with the Folin phenol reagent.

Authors:  O H LOWRY; N J ROSEBROUGH; A L FARR; R J RANDALL
Journal:  J Biol Chem       Date:  1951-11       Impact factor: 5.157

3.  Biochemical discrimination of Hurler and Scheie syndromes.

Authors:  J J Hopwood; V Muller
Journal:  Clin Sci (Lond)       Date:  1979-09       Impact factor: 6.124

4.  Allelism, non-allelism, and genetic compounds among the mucopolysaccharidoses.

Authors:  V A McKusick; R R Howell; I E Hussels; E F Neufeld; R E Stevenson
Journal:  Lancet       Date:  1972-05-06       Impact factor: 79.321

5.  Hurler's syndrome, an -L-iduronidase deficiency.

Authors:  R Matalon; A Dorfman
Journal:  Biochem Biophys Res Commun       Date:  1972-05-26       Impact factor: 3.575

6.  Studies on the alpha-L-iduronidase activity of beta-glucuronidase preparations from bovine liver, rat liver, and rat preputial gland.

Authors:  H Kosaka; M Isemura; T Ono; Y Nishimura; K Kato
Journal:  J Biochem       Date:  1980-07       Impact factor: 3.387

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

Authors:  M Isemura; H Kosaka; T Ikenaka; R Kido; T Yoshimura
Journal:  J Biochem       Date:  1978-09       Impact factor: 3.387

8.  Hurler-Scheie phenotype: a report of two pairs of inbred sibs.

Authors:  N Kaibara; M Eguchi; K Shibata; K Takagishi
Journal:  Hum Genet       Date:  1979       Impact factor: 4.132

9.  A fluorometric assay using 4-methylumbelliferyl alpha-L-iduronide for the estimation of alpha-L-iduronidase activity and the detection of Hurler and Scheie syndromes.

Authors:  J J Hopwood; V Muller; A Smithson; N Baggett
Journal:  Clin Chim Acta       Date:  1979-03-01       Impact factor: 3.786

10.  The defect in the Hurler and Scheie syndromes: deficiency of -L-iduronidase.

Authors:  G Bach; R Friedman; B Weissmann; E F Neufeld
Journal:  Proc Natl Acad Sci U S A       Date:  1972-08       Impact factor: 11.205
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  3 in total

1.  Hurler-Scheie phenotype associated with consanguinity.

Authors:  D L Davies; G N Dutton; J Farquharson; R W Logan; J L Tolmie
Journal:  J Inherit Metab Dis       Date:  1989       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

3.  Immunopurification and characterization of human alpha-L-iduronidase with the use of monoclonal antibodies.

Authors:  P R Clements; D A Brooks; P A McCourt; J J Hopwood
Journal:  Biochem J       Date:  1989-04-01       Impact factor: 3.857
  3 in total

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