Effect of transgene copy number on survival in the G93A SOD1 transgenic mouse model of ALS.
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.
Alexander
Guillermo M
GM
Department of Neurology, College of Medicine, Drexel University, Mail stop 423, 245 North 15th Street, Philadelphia, PA 19102, USA. guillermo.alexander@drexel.edu
Erwin
Kirsten L
KL
Byers
Nathaniel
N
Deitch
Jeffrey S
JS
Augelli
Brian J
BJ
Blankenhorn
Elizabeth P
EP
Heiman-Patterson
Terry D
TD
D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics*; D000818:Animals; D004195:Disease Models, Animal; D018628:Gene Dosage; D006801:Humans; D007376:Interleukin-2 / Q000235:genetics / Q000378:metabolism; D051379:Mice; D008822:Mice, Transgenic; D010641:Phenotype; D012333:RNA, Messenger / Q000378:metabolism; D011995:Recombination, Genetic / Q000502:physiology; D020133:Reverse Transcriptase Polymerase Chain Reaction / Q000379:methods; D013482:Superoxide Dismutase / Q000235:genetics* / Q000378:metabolism; D013534:Survival / Q000502:physiology; D019076:Transgenes / Q000502:physiology*
D007376:Interleukin-2; D012333:RNA, Messenger; C109415:SOD1 G93A protein; D013482:Superoxide Dismutase