Article
Metabolic signatures of amyotrophic lateral sclerosis reveal insights into disease pathogenesis.
Proceedings of the National Academy of Sciences of the United States of America - 25 Jun 2013
Dodge James C, Treleaven Christopher M, Fidler Jonathan A, Tamsett Thomas J, Bao Channa, Searles Michelle, Taksir Tatyana V, Misra Kuma, Sidman Richard L, Cheng Seng H, Shihabuddin Lamya S
Abstract excerpt
Metabolic dysfunction is an important modulator of disease course in amyotrophic lateral sclerosis (ALS). We report here that a familial mouse model (transgenic mice over-expressing the G93A mutation of the Cu/Zn superoxide dismutase 1 gene) of ALS enters a progressive state of acidosis that is associated with several metabolic (hormonal) alternations that favor lipolysis. Extensive investigation of the major...
Topics
- Acidosis
- Amyotrophic Lateral Sclerosis
- Animals
- Disease Models, Animal
- Disease Progression
- Glycogen
- Humans
- Mice
- Mice, Transgenic
- Mutation