Researchers Create First ALS, Dementia Mouse Model

Researchers have created the first animal model of ALS dementia--a form of ALS that also damages the brain. Researchers at Northwestern Medicine have developed the model to directly examine the brains of living mice, under anesthesia, at the microscopic level.

"This new model will allow rapid testing and direct monitoring of drugs in real time," said Northwestern scientist and study senior author Teepu Siddique, M.D., in a news release. "This will allow scientists to move quickly and accelerate the testing of drug therapies."

The new model shows the pathological hallmarks of the disease in humans with mutations in the genes of UBQLN2 (ubliqulin 2) and SQSTM1 (P62) that Siddique and colleagues identified in 2011. That pathology was linked to all forms of ALS and ALS/dementia.

Furthermore, researchers said they have have reproduced behavioral, neurophysiological and pathological changes in a mouse that mimic this form of dementia associated with ALS (amyotrophic lateral sclerosis).

"ALS with dementia is an even more vicious disease than ALS alone because it attacks the brain causing changes in behavior and language well as paralysis," he concluded.

As it's estimated that five percent or more of ALS cases also have Lou Gehrig's Disease, also known as ALS/dementia, researchers are continuing to monitor drugs that could potentially help the health issue in the future. 

More information regarding the findings can be seen via the Proceedings of the National Academy of Sciences (PNAS).