Literature DB >> 41974

alpha-Glucosidase in Pompe's disease.

D M Broadhead, J Butterworth.   

Abstract

Isoelectric precipitation at pH 5.0 and the use of the inhibitors, turanose, maltose and citrate, enabled the diagnosis of Pompe's disease to be made in dextran-isolated leucocytes using 4-methylumbelliferyl-alpha-D-glucopyranoside as substrate. These techniques were unnecessary with lymphocytes as the deficiency of acid alpha-glucosidase could be shown directly.

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Year:  1978        PMID： 41974     DOI： 10.1007/bf01805584

Source DB:  PubMed          Journal:  J Inherit Metab Dis        ISSN： 0141-8955            Impact factor:   4.982


  8 in total

1.  DIAGNOSIS OF GENERALIZED GLYCOGEN STORAGE DISEASE (POMPE'S DISEASE).

Authors:  F HUIJING; G LOSEKOOT
Journal:  J Pediatr       Date:  1963-11       Impact factor: 4.406

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.  Physico-chemical and immunological properties of acid alpha-glucosidase from various human tissues in relation to glycogenosis type II (Pompe's disease).

Authors:  J F Koster; R G Slee; J M Van der Klei-Van Moorsel; P J Rietra; C J Lucas
Journal:  Clin Chim Acta       Date:  1976-04-01       Impact factor: 3.786

4.  Pompe's disease: diagnosis in kidney and leucocytes using 4-methylumbelliferyl-alpha-D-glucopyranoside.

Authors:  D M Broadhead; J Butterworth
Journal:  Clin Genet       Date:  1978-06       Impact factor: 4.438

5.  A fluorometric assay of alpha-glucosidase and its application in the study of Pompe's disease.

Authors:  I S Salafsky; H L Nadler
Journal:  J Lab Clin Med       Date:  1973-03

6.  Isolation of mononuclear cells and granulocytes from human blood. Isolation of monuclear cells by one centrifugation, and of granulocytes by combining centrifugation and sedimentation at 1 g.

Authors:  A Böyum
Journal:  Scand J Clin Lab Invest Suppl       Date:  1968

7.  The use of leucocytes as an aid in the diagnosis of glycogen storage disease type II (Pompe's disease).

Authors:  J F Koster; R G Slee; W C Hülsmann
Journal:  Clin Chim Acta       Date:  1974-03-26       Impact factor: 3.786

Review 8.  The use of white cells as a source of diagnostic material for lipid storage diseases.

Authors:  R A Snyder; R O Brady
Journal:  Clin Chim Acta       Date:  1969-08       Impact factor: 3.786
  8 in total
  1 in total

1.  Enhanced delivery of α-glucosidase for Pompe disease by ICAM-1-targeted nanocarriers: comparative performance of a strategy for three distinct lysosomal storage disorders.

Authors:  Janet Hsu; Laura Northrup; Tridib Bhowmick; Silvia Muro
Journal:  Nanomedicine       Date:  2011-09-09       Impact factor: 5.307
  1 in total

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