/// Alzheimer’s disease
Alzheimer’s disease is characterized by the presence in the brain of senile plaques, mainly due to the progressive accumulation and deposition of a 40/42 amino-acid long b-amyloid protein. The model mimics this pathological feature. The smallest endogenously identified fragment, carrying the aggregation and toxic properties of the whole protein, is centrally injected in mice or rats. Morphological, biochemical and behavioral signs of amyloid toxicity develop within 1 or 2 weeks.
Suitable to examine the anti-amnesic or neuroprotective activity of ligands :
/// Amyotrophic Lateral Sclerosis (ALS)
SOD1 transgenic mice are used as a model for ALS. These mice have the mutant human SOD1 (G93A) substitution. Motor impairment is first observed at 12 weeks of age and includes changes in body weight, decline in grip strength and general motor activity, which can be assessed using the tests outlined below. This phenotype continues to decline until death at an average age of 19 weeks.
/// Huntington’s Disease (HD)
The R6/2 transgenic mouse model was the first model developed (Mangiarini et al., 1996) and it is widely used to understand the pathogenesis of the disease as well as for the evaluation of new therapies. These mice present severe weight loss, progressive motor, cognitive and psychiatric deficits.