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Researchers Develop First Mouse Model For ALS Dementia

Update Date: Sep 25, 2014 08:54 AM EDT

Researchers have developed the first animal model for ALS dementia - a form of ALS that also damages the brain. The advance would help researchers see the brains of living mice, under anesthesia, at the microscopic level. 

The development would also allow direct monitoring of test drugs to determine if they work. 

"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 the press release. "This will allow scientists to move quickly and accelerate the testing of drug therapies."

The new mouse model has the pathological hallmarks of the disease in humans with mutations in the genes for UBQLN2 (ubliqulin 2) and SQSTM1 (P62) that Siddique and colleagues identified in 2011, added the press release. The same pathology has also been linked to all forms of ALS and ALS/dementia. 

Siddique and Han-Xiang Deng, M.D., the corresponding authors on the paper, said they have reproduced behavioral, neurophysiological and pathological changes in a mouse that mimic this form of dementia associated with ALS (amyotrophic lateral sclerosis).

Researchers also noted that five percent or more of ALS cases, also known as Lou Gehrig's Disease also have ALS/dementia. 

"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," Siddique said in the press release. 

The study was published in the Proceedings of the National Academy of Sciences.

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