Literature DB >> 3707714

Occurrence of lysoganglioside lyso-GM2 (II3-Neu5Ac-gangliotriaosylsphingosine) in GM2 gangliosidosis brain.

S Neuenhofer, E Conzelmann, G Schwarzmann, H Egge, K Sandhoff.   

Abstract

Lysoganglioside lyso-GM2 (sialylgangliotriaosylsphingosine) was detected in a brain sample of a patient with variant B of infantile GM2 gangliosidosis (Tay-Sachs disease) at a concentration of approx. 15 nmol/g wet tissue. Neither lysoganglioside lyso-GM2 nor the corresponding GM1 derivative could be detected in normal brain.

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Year:  1986        PMID： 3707714     DOI： 10.1515/bchm3.1986.367.1.241

Source DB:  PubMed          Journal:  Biol Chem Hoppe Seyler        ISSN： 0177-3593


  21 in total

1.  A novel assay method for glycosphingolipid deacylase by enzyme-linked immunochemical detection of lysoglycosphingolipid.

Authors:  K Izumi; M T Sawada
Journal:  Lipids       Date:  2001-01       Impact factor: 1.880

2.  Effective gene therapy in an authentic model of Tay-Sachs-related diseases.

Authors:  M Begoña Cachón-González; Susan Z Wang; Andrew Lynch; Robin Ziegler; Seng H Cheng; Timothy M Cox
Journal:  Proc Natl Acad Sci U S A       Date:  2006-06-26       Impact factor: 11.205

Review 3.  Twenty five years of the "psychosine hypothesis": a personal perspective of its history and present status.

Authors:  K Suzuki
Journal:  Neurochem Res       Date:  1998-03       Impact factor: 3.996

4.  Partial synthesis of ganglioside and lysoganglioside lipoforms as internal standards for MS quantification.

Authors:  Martin Gantner; Günter Schwarzmann; Konrad Sandhoff; Thomas Kolter
Journal:  J Lipid Res       Date:  2014-10-22       Impact factor: 5.922

5.  Bone marrow transplantation prolongs life span and ameliorates neurologic manifestations in Sandhoff disease mice.

Authors:  F Norflus; C J Tifft; M P McDonald; G Goldstein; J N Crawley; A Hoffmann; K Sandhoff; K Suzuki; R L Proia
Journal:  J Clin Invest       Date:  1998-05-01       Impact factor: 14.808

6.  Protease-resistant modified human β-hexosaminidase B ameliorates symptoms in GM2 gangliosidosis model.

Authors:  Keisuke Kitakaze; Yasumichi Mizutani; Eiji Sugiyama; Chikako Tasaki; Daisuke Tsuji; Nobuo Maita; Takatsugu Hirokawa; Daisuke Asanuma; Mako Kamiya; Kohei Sato; Mitsutoshi Setou; Yasuteru Urano; Tadayasu Togawa; Akira Otaka; Hitoshi Sakuraba; Kohji Itoh
Journal:  J Clin Invest       Date:  2016-03-28       Impact factor: 14.808

Review 7.  Pathology and current treatment of neurodegenerative sphingolipidoses.

Authors:  Matthias Eckhardt
Journal:  Neuromolecular Med       Date:  2010-08-22       Impact factor: 3.843

Review 8.  Glycosphingolipid degradation and animal models of GM2-gangliosidoses.

Authors:  T Kolter; K Sandhoff
Journal:  J Inherit Metab Dis       Date:  1998-08       Impact factor: 4.982

9.  Sphingosylphosphorylcholine in Niemann-Pick disease brain: accumulation in type A but not in type B.

Authors:  C Rodriguez-Lafrasse; M T Vanier
Journal:  Neurochem Res       Date:  1999-02       Impact factor: 3.996

Review 10.  [Sphingolipid storage diseases of the central nervous system: bases of biochemical and clinical heterogeneity].

Authors:  K Sandhoff; L Quintern
Journal:  Naturwissenschaften       Date:  1988-03
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