Literature DB >> 9071485

Neurological manifestations of knockout mice with beta-galactosidase deficiency.

J Matsuda1, O Suzuki, A Oshima, A Ogura, M Naiki, Y Suzuki.   

Abstract

We succeeded in producing the beta-galactosidase-deficient knockout mouse by gene targeting in embryonic stem cells. The mutant mice developed progressive spastic diplegia within a few months after birth, and died of emaciation at 7-10 months of age. This is an authentic murine model of human GMI-gangliosidosis, and is useful for studies of its pathogenesis and treatment.

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Year:  1997        PMID： 9071485     DOI： 10.1016/s0387-7604(96)00077-0

Source DB:  PubMed          Journal:  Brain Dev        ISSN： 0387-7604            Impact factor:   1.961


  12 in total

Review 1.  The GM1 and GM2 Gangliosidoses: Natural History and Progress toward Therapy.

Authors:  Debra S Regier; Richard L Proia; Alessandra D'Azzo; Cynthia J Tifft
Journal:  Pediatr Endocrinol Rev       Date:  2016-06

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.  Systemic AAV9 gene transfer in adult GM1 gangliosidosis mice reduces lysosomal storage in CNS and extends lifespan.

Authors:  Cara M Weismann; Jennifer Ferreira; Allison M Keeler; Qin Su; Linghua Qui; Scott A Shaffer; Zuoshang Xu; Guangping Gao; Miguel Sena-Esteves
Journal:  Hum Mol Genet       Date:  2015-05-10       Impact factor: 6.150

4.  Beta-galactosidase-deficient mouse as an animal model for GM1-gangliosidosis.

Authors:  J Matsuda; O Suzuki; A Oshima; A Ogura; Y Noguchi; Y Yamamoto; T Asano; K Takimoto; K Sukegawa; Y Suzuki; M Naiki
Journal:  Glycoconj J       Date:  1997-09       Impact factor: 2.916

Review 5.  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

Review 6.  Animal models of lysosomal disease: an overview.

Authors:  K Suzuki; J E Månsson
Journal:  J Inherit Metab Dis       Date:  1998-08       Impact factor: 4.982

7.  A symptomatic Fabry disease mouse model generated by inducing globotriaosylceramide synthesis.

Authors:  Atsumi Taguchi; Hiroki Maruyama; Masaaki Nameta; Tadashi Yamamoto; Junichiro Matsuda; Ashok B Kulkarni; Hidekatsu Yoshioka; Satoshi Ishii
Journal:  Biochem J       Date:  2013-12-15       Impact factor: 3.857

8.  Molecular consequences of the pathogenic mutation in feline GM1 gangliosidosis.

Authors:  Douglas R Martin; Brigitte A Rigat; Polly Foureman; G S Varadarajan; Misako Hwang; Barbara K Krum; Bruce F Smith; John W Callahan; Don J Mahuran; Henry J Baker
Journal:  Mol Genet Metab       Date:  2008-03-18       Impact factor: 4.797

9.  Myelin abnormalities in the optic and sciatic nerves in mice with GM1-gangliosidosis.

Authors:  Karie A Heinecke; Adrienne Luoma; Alessandra d'Azzo; Daniel A Kirschner; Thomas N Seyfried
Journal:  ASN Neuro       Date:  2015-02-18       Impact factor: 4.146

10.  Axonopathy and Reduction of Membrane Resistance: Key Features in a New Murine Model of Human GM1-Gangliosidosis.

Authors:  Deborah Eikelberg; Annika Lehmbecker; Graham Brogden; Witchaya Tongtako; Kerstin Hahn; Andre Habierski; Julia B Hennermann; Hassan Y Naim; Felix Felmy; Wolfgang Baumgärtner; Ingo Gerhauser
Journal:  J Clin Med       Date:  2020-04-02       Impact factor: 4.241
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