Shiverer: an autosomal recessive mutant mouse with myelin deficiency
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The Journal of Heredity
|March 1, 1981
Abstract:
Shiverer is an autosomal recessive trait in the mouse characterized by early generalized tremors that become prominent in the hindquarters with age. Seizure behavior begins after weaning and increases in frequency during the animal's shortened lifespan. The most prominent pathological feature is a deficiency of myelin and myelin basic protein in the central nervous system.
Insights
The Shiverer mouse model exhibits tremors and seizures due to a myelin deficiency in the central nervous system. This genetic trait impacts myelin basic protein, leading to neurological dysfunction and a shortened lifespan.
Area of Science:
- Neuroscience
- Genetics
- Animal Models
Background:
- Shiverer is an autosomal recessive mutation in mice.
- It is characterized by neurological deficits including tremors and seizures.
- Affected animals have a shortened lifespan.
Purpose of the Study:
- To investigate the neurological and pathological characteristics of the Shiverer mouse model.
- To understand the genetic basis of myelin deficiency in this model.
Main Methods:
- Observation of tremor and seizure behavior.
- Assessment of myelin and myelin basic protein levels in the central nervous system.
Main Results:
- Shiverer mice display generalized tremors, worsening with age.
- Seizure activity increases post-weaning, correlating with lifespan reduction.
- A significant deficiency in central nervous system myelin and myelin basic protein is observed.
Conclusions:
- The Shiverer mutation results in severe myelin defects in the central nervous system.
- This myelin deficiency underlies the observed neurological symptoms and reduced lifespan.
- The Shiverer mouse serves as a valuable model for studying myelin disorders.