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

被引：140

作者：

Alexander, GM

Erwin, KL

Byers, N

Deitch, JS

Augelli, BJ

Blankenhorn, EP

Heiman-Patterson, TD

机构：

[1] Drexel Univ, Coll Med, Dept Neurol, Philadelphia, PA 19102 USA

[2] Drexel Univ, Coll Med, Dept Microbiol & Immunol, Philadelphia, PA 19102 USA

[3] Temple Univ, Sch Med, Dept Neurol, Philadelphia, PA 19140 USA

来源：

MOLECULAR BRAIN RESEARCH | 2004年 / 130卷 / 1-2期

关键词：

SOD1; ALS; real time PCR; transgene copy number; G93A; mice;

D O I：

10.1016/j.molbrainres.2004.07.002

中图分类号：

Q189 [神经科学];

学科分类号：

071006 [神经生物学];

摘要：

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 SOD I mouse model of ALS. (C) 2004 Elsevier B.V. All rights reserved.

引用

页码：7 / 15

页数：9

共 19 条

[1]

Elevated cortical extracellular fluid glutamate in transgenic mice expressing human mutant (G93A) Cu/Zn superoxide dismutase [J].