Literature DB >> 2706492

The twitcher mouse: accumulation of galactosylsphingosine and pathology of the central nervous system.

K Tanaka1, H Nagara, T Kobayashi, I Goto.   

Abstract

In the twitcher mouse, a murine model of globoid cell leukodystrophy (GLD), pathological changes of various parts of the central nervous system correlated well with the concentration of galactosylsphingosine (psychosine). The development of GLD lesions was more obvious in tracts with a more rapid progression of myelination. It was suggested that accumulation of galactosylsphingosine subsequent to myelin maturation caused suicidal death of myelin forming cells.

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Year:  1989        PMID： 2706492     DOI： 10.1016/0006-8993(89)91198-0

Source DB:  PubMed          Journal:  Brain Res        ISSN： 0006-8993            Impact factor:   3.252


  9 in total

1.  Effect of vitamin D3 intake on the onset of disease in a murine model of human Krabbe disease.

Authors:  Manjeet K Paintlia; Inderjit Singh; Avtar K Singh
Journal:  J Neurosci Res       Date:  2014-09-19       Impact factor: 4.164

Review 2.  Multi-system disorders of glycosphingolipid and ganglioside metabolism.

Authors:  You-Hai Xu; Sonya Barnes; Ying Sun; Gregory A Grabowski
Journal:  J Lipid Res       Date:  2010-03-08       Impact factor: 5.922

3.  Long-Term Improvement of Neurological Signs and Metabolic Dysfunction in a Mouse Model of Krabbe's Disease after Global Gene Therapy.

Authors:  Michael S Marshall; Yazan Issa; Benas Jakubauskas; Monika Stoskute; Vince Elackattu; Jeffrey N Marshall; Wil Bogue; Duc Nguyen; Zane Hauck; Emily Rue; Subha Karumuthil-Melethil; Violeta Zaric; Maarten Bosland; Richard B van Breemen; Maria I Givogri; Steven J Gray; Stephen J Crocker; Ernesto R Bongarzone
Journal:  Mol Ther       Date:  2018-01-17       Impact factor: 11.454

4.  The Spectrum of Krabbe Disease in Greece: Biochemical and Molecular Findings.

Authors:  Evangelia Dimitriou; Monica Cozar; Irene Mavridou; Daniel Grinberg; Lluïsa Vilageliu; Helen Michelakakis
Journal:  JIMD Rep       Date:  2015-06-25

5.  Missense mutation in mouse GALC mimics human gene defect and offers new insights into Krabbe disease.

Authors:  Gregory B Potter; Marta Santos; Muriel T Davisson; David H Rowitch; Dan L Marks; Ernesto R Bongarzone; Magdalena A Petryniak
Journal:  Hum Mol Genet       Date:  2013-04-24       Impact factor: 6.150

6.  Generation of a LacZ reporter transgenic mouse line for the stereological analysis of oligodendrocyte loss in galactosylceramidase deficiency.

Authors:  Hongling Zhu; Francesca Ornaghi; Sophie Belin; Maria I Givogri; Lawrence Wrabetz; Ernesto R Bongarzone
Journal:  J Neurosci Res       Date:  2016-07-18       Impact factor: 4.164

7.  Waning efficacy in a long-term AAV-mediated gene therapy study in the murine model of Krabbe disease.

Authors:  Gregory J Heller; Michael S Marshall; Yazan Issa; Jeffrey N Marshall; Duc Nguyen; Emily Rue; Koralege C Pathmasiri; Miriam S Domowicz; Richard B van Breemen; Leon M Tai; Stephanie M Cologna; Stephen J Crocker; Maria I Givogri; Mark S Sands; Ernesto R Bongarzone
Journal:  Mol Ther       Date:  2021-01-26       Impact factor: 11.454

8.  Accumulation of lysosulfatide in the brain of arylsulfatase A-deficient mice.

Authors:  Maria Blomqvist; Volkmar Gieselmann; Jan-Eric Månsson
Journal:  Lipids Health Dis       Date:  2011-02-07       Impact factor: 3.876

Review 9.  Developing therapeutic approaches for metachromatic leukodystrophy.

Authors:  Shilpa A Patil; Gustavo H B Maegawa
Journal:  Drug Des Devel Ther       Date:  2013-08-08       Impact factor: 4.162
  9 in total

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