Northwestern Medicine scientists have reportedly developed an animal model for ALS dementia. A university news release notes that the research findings will allow researchers to directly see the brains of living mice, under anesthesia, at the microscopic level. This will also allow for direct observation of test treatments to determine if they work.

Teepu Siddique, MD, senior study author, Northwestern scientist, states in the release that the new mouse model will allow for rapid testing and direct monitoring of treatments in real time.

“This will allow scientists to move quickly and accelerate the testing of drug therapies,” Siddique adds.

According to the release, the new mouse model has the pathological traits of the disease in humans with mutations in the genes for UBQLN2 (ubliqulin 2) and SQSTM1 (P62) that Siddique and colleagues pinpointed in 2011. That pathology was linked to all forms of ALS and ALS/dementia, the release says.

Siddique and corresponding author Han-Xian Deng, MD, note in the release that they have reproduced behavioral, neurophysiological, and pathological changes in a mouse that mimic this form of dementia linked to ALS.

Siddique explains that it has been a challenge for scientists to reproduce the genetic mutations of ALS, particularly ALS/dementia in animal models, and in turn this has slowed drug therapy testing.

The release reports that 5% or more of ALS cases also have ALS/dementia, which Siddique says “is an even more vicious disease than ALS alone because it attacks the brain, causing changes in behavior and language, as well as paralysis.”

Photo Credit: Northwestern University

Source: Northwestern University