Literature DB >> 15519671

Effect of transgene copy number on survival in the G93A SOD1 transgenic mouse model of ALS.

Guillermo M Alexander1, Kirsten L Erwin, Nathaniel Byers, Jeffrey S Deitch, Brian J Augelli, Elizabeth P Blankenhorn, Terry D Heiman-Patterson.   

Abstract

Transgenic mice expressing multiple copies of the G93A mutant form of SOD1 develop motor neuron pathology and clinical symptoms similar to those seen in patients with amyotrophic lateral sclerosis (ALS). The phenotype of these mice is dependent on the number of transgene copies in their genome. Changes in transgene copy number, although rare, can sometimes occur while mating due to intra locus recombination events during meiosis. The objective of this study was to develop a real time quantitative PCR method to determine changes in transgene copy number in these mice and to evaluate the effect of transgene copy number on the phenotype of the G93A SOD1 mouse model of ALS.

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Year:  2004        PMID: 15519671     DOI: 10.1016/j.molbrainres.2004.07.002

Source DB:  PubMed          Journal:  Brain Res Mol Brain Res        ISSN: 0169-328X


  60 in total

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Authors:  Tennore Ramesh; Alison N Lyon; Ricardo H Pineda; Chunping Wang; Paul M L Janssen; Benjamin D Canan; Arthur H M Burghes; Christine E Beattie
Journal:  Dis Model Mech       Date:  2010-05-26       Impact factor: 5.758

2.  Calpastatin inhibits motor neuron death and increases survival of hSOD1(G93A) mice.

Authors:  Mala V Rao; Jabbar Campbell; Arti Palaniappan; Asok Kumar; Ralph A Nixon
Journal:  J Neurochem       Date:  2016-03-23       Impact factor: 5.372

3.  Application of a bacterial artificial chromosome modification system for a human artificial chromosome vector.

Authors:  Shigeyuki Yamaguchi; Ryosuke Niwa; Yasuhiro Kazuki; Tetsuya Ohbayashi
Journal:  Yonago Acta Med       Date:  2011-03-01       Impact factor: 1.641

4.  Diapocynin and apocynin administration fails to significantly extend survival in G93A SOD1 ALS mice.

Authors:  Kari A Trumbull; Donna McAllister; Mandi M Gandelman; Whitney Y Fung; Thomas Lew; Lucy Brennan; Nathan Lopez; Jeff Morré; Balaraman Kalyanaraman; Joseph S Beckman
Journal:  Neurobiol Dis       Date:  2011-07-28       Impact factor: 5.996

5.  The UPR-PERK pathway is not a promising therapeutic target for mutant SOD1-induced ALS.

Authors:  Yulia Dzhashiashvili; Chase P Monckton; Harini S Shah; Rejani B Kunjamma; Brian Popko
Journal:  Neurobiol Dis       Date:  2019-03-26       Impact factor: 5.996

6.  Axonal transport deficits and degeneration can evolve independently in mouse models of amyotrophic lateral sclerosis.

Authors:  Petar Marinkovic; Miriam S Reuter; Monika S Brill; Leanne Godinho; Martin Kerschensteiner; Thomas Misgeld
Journal:  Proc Natl Acad Sci U S A       Date:  2012-02-27       Impact factor: 11.205

7.  Regulation of Intracellular Copper by Induction of Endogenous Metallothioneins Improves the Disease Course in a Mouse Model of Amyotrophic Lateral Sclerosis.

Authors:  Eiichi Tokuda; Shunsuke Watanabe; Eriko Okawa; Shin-ichi Ono
Journal:  Neurotherapeutics       Date:  2015-04       Impact factor: 7.620

8.  Loss of ALS2/Alsin exacerbates motor dysfunction in a SOD1-expressing mouse ALS model by disturbing endolysosomal trafficking.

Authors:  Shinji Hadano; Asako Otomo; Ryota Kunita; Kyoko Suzuki-Utsunomiya; Akira Akatsuka; Masato Koike; Masashi Aoki; Yasuo Uchiyama; Yasuto Itoyama; Joh-E Ikeda
Journal:  PLoS One       Date:  2010-03-22       Impact factor: 3.240

9.  Reticulon-4A (Nogo-A) redistributes protein disulfide isomerase to protect mice from SOD1-dependent amyotrophic lateral sclerosis.

Authors:  Yvonne S Yang; Noam Y Harel; Stephen M Strittmatter
Journal:  J Neurosci       Date:  2009-11-04       Impact factor: 6.167

10.  No benefit from chronic lithium dosing in a sibling-matched, gender balanced, investigator-blinded trial using a standard mouse model of familial ALS.

Authors:  Alan Gill; Joshua Kidd; Fernando Vieira; Kenneth Thompson; Steven Perrin
Journal:  PLoS One       Date:  2009-08-03       Impact factor: 3.240

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