Literature DB >> 8051915

High-resolution loading tests in the study of genetic heterogeneity in gangliosidosis fibroblasts.

V S Akhunov1, T V Mirenburg, X D Krasnopolskaya.   

Abstract

GM1- and GM2-gangliosides were isolated from brain and radiolabelled. The labelled moieties were localized by hydrolysis with lysosomal enzymes, followed by thin-layer chromatography of the products. High-resolution loading tests with labelled gangliosides were developed and found to differentiate infantile and juvenile forms of GM1- and GM2-gangliosidoses as well as the identification of B, O and AB types of GM2-gangliosidosis.

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Year:  1994        PMID： 8051915     DOI： 10.1007/bf00735405

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


  21 in total

1.  Characterization of the cellular binding of exogenous gangliosides.

Authors:  R Callies; G Schwarzmann; K Radsak; R Siegert; H Wiegandt
Journal:  Eur J Biochem       Date:  1977-11-01

2.  Internalization of exogenous gangliosides in cultured skin fibroblasts for the diagnosis of mucolipidosis IV.

Authors:  M Zeigler; G Bach
Journal:  Clin Chim Acta       Date:  1986-06-15       Impact factor: 3.786

3.  The accumulation of cerebroside sulfates by fibroblasts in culture from patients with late infantile metachromatic leukocystrophy.

Authors:  M T Porter; A L Fluharty; S E Harris; H Kihara
Journal:  Arch Biochem Biophys       Date:  1970-06       Impact factor: 4.013

4.  Diagnosis and prevention of lysosomal storage diseases in Russia.

Authors:  K D Krasnopolskaya; T V Mirenburg; E L Aronovich; T V Lebedeva; O N Odinokova; N A Demina; V M Kozlova; M I Kuznetsov
Journal:  J Inherit Metab Dis       Date:  1993       Impact factor: 4.982

5.  Metabolism of sphingomyelin by intact cultured fibroblasts: differentiation of Niemann-Pick disease type A and B.

Authors:  A L Beaudet; A A Manschreck
Journal:  Biochem Biophys Res Commun       Date:  1982-03-15       Impact factor: 3.575

6.  Role of lysosomal acid ceramidase in the metabolism of ceramide in human skin fibroblasts.

Authors:  W W Chen; A B Moser; H W Moser
Journal:  Arch Biochem Biophys       Date:  1981-05       Impact factor: 4.013

7.  [Identification of GM2-gangliosidosis using an in vitro loading test].

Authors:  V S Akhunov; K D Krasnopol'skaia
Journal:  Vopr Med Khim       Date:  1987 Jul-Aug

8.  The nature of mannose-containing material which accumulates in cultured fibroblasts from patients with mannosidosis.

Authors:  A Chester; B Hultberg; E Nordén N; L Szabó
Journal:  Biochim Biophys Acta       Date:  1980-02-07

9.  GM2-ganglioside metabolism in cultured human skin fibroblasts: unambiguous diagnosis of GM2-gangliosidosis.

Authors:  S Raghavan; A Krusell; T A Lyerla; E G Bremer; E H Kolodny
Journal:  Biochim Biophys Acta       Date:  1985-04-25

10.  A simple and novel method for tritium labeling of gangliosides and other sphingolipids.

Authors:  G Schwarzmann
Journal:  Biochim Biophys Acta       Date:  1978-04-28
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