Literature DB >> 421348

The assay of sphingolipid hydrolases in white blood cells with labelled natural substrates.

L Svennerholm, G Håkansson, J E Månsson, M T Vanier.   

Abstract

Methods were elaborated for the assay on leukocytes of sphingolipid hydrolases with native and artificial substrates. The optimal conditions for the isolation of leukocytes and the preparation of labelled sphingolipids were investigated. The KM and Vmax values for all substrates are given as the specific activities of the sphingolipidhydrolases in normal males and females. It is concluded that leukocytes are the enzyme source of choice for the enzymic diagnosis of neurolipidoses.

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Year:  1979        PMID： 421348     DOI： 10.1016/0009-8981(79)90396-6

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


  14 in total

1.  A new fluorimetric enzyme assay for the diagnosis of Niemann-Pick A/B, with specificity of natural sphingomyelinase substrate.

Authors:  O P van Diggelen; Ya V Voznyi; J L M Keulemans; K Schoonderwoerd; J Ledvinova; E Mengel; M Zschiesche; R Santer; K Harzer
Journal:  J Inherit Metab Dis       Date:  2005       Impact factor: 4.982

2.  Effect of single-nucleotide polymorphisms of the 5' untranslated region of the human α-galactosidase gene on enzyme activity, and their frequencies in Portuguese caucasians.

Authors:  J P Oliveira; S Ferreira; J Barceló; P Gaspar; F Carvalho; M C Sá Miranda; J-E Månsson
Journal:  J Inherit Metab Dis       Date:  2008-11-03       Impact factor: 4.982

3.  Hexosaminidase assays.

Authors:  Michaela Wendeler; Konrad Sandhoff
Journal:  Glycoconj J       Date:  2009-11       Impact factor: 2.916

4.  Ganglioside GM1 metabolism in living human fibroblasts with beta-galactosidase deficiency.

Authors:  G M Mancini; A T Hoogeveen; H Galjaard; J E Mansson; L Svennerholm
Journal:  Hum Genet       Date:  1986-05       Impact factor: 4.132

5.  Screening of degradative enzymes from articular cartilage in experimental osteoarthritis.

Authors:  E Vignon; O Gateau; A Martin; D Hartmann; J Bejui; M C Biol; M T Vanier; P Louisot; M Richard
Journal:  Clin Rheumatol       Date:  1987-06       Impact factor: 2.980

6.  A family with visceral course of Niemann-Pick disease, macular halo syndrome and low sphingomyelin degradation rate.

Authors:  W Sperl; G Bart; M T Vanier; H Christomanou; I Baldissera; E Steichen-Gersdorf; E Paschke
Journal:  J Inherit Metab Dis       Date:  1994       Impact factor: 4.982

7.  Biochemical, genetic, psychometric, and neuropsychological studies in heterozygotes of a family with globoid cell leucodystrophy (Krabbe's disease).

Authors:  H Christomanou; S Jaffé; J Martinius; C Cáp; K Betke
Journal:  Hum Genet       Date:  1981       Impact factor: 4.132

8.  [Lipid-protein interactions: mechanisms of enzymatic glycolipid catabolism and their genetic restrictive escapes].

Authors:  K Sandhoff
Journal:  Naturwissenschaften       Date:  1980-09

9.  Sulfatide is associated with insulin granules and located to microdomains of a cultured beta cell line.

Authors:  Maria Blomqvist; Thomas Osterbye; Jan-Eric Månsson; Thomas Horn; Karsten Buschard; Pam Fredman
Journal:  Glycoconj J       Date:  2002-07       Impact factor: 2.916

10.  The g.1170C>T polymorphism of the 5' untranslated region of the human alpha-galactosidase gene is associated with decreased enzyme expression--evidence from a family study.

Authors:  J P Oliveira; S Ferreira; C Reguenga; F Carvalho; J-E Månsson
Journal:  J Inherit Metab Dis       Date:  2008-11-03       Impact factor: 4.982
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