From the lab of Dr. P. Hande Ozdinler, a researcher at the Les Turner ALS Research and Patient Center at Northwestern Medicine, comes a breakthrough in ALS research.
Research performed by Javier Jara, Ph.D. and Baris Genc, Ph.D., under the direction of Dr. Ozdinler have found that the loss of a gene linked to Parkinson’s disease exposes motor neurons in the brain to increased stress and leads to neurodegeneration. The study is published in the journal Cerebral Cortex.
The researchers created a mouse model that lacked a gene, called UCHL1, which has been identified as a rare cause of Parkinson’s disease and is characterized by loss of upper motor neurons. Upper motor neurons link the brain and spinal cord and are also lost in ALS.
From studying the mice lacking the gene, the researchers found selective vulnerability of upper motor neurons. Degeneration of these neurons was accompanied by signs of stress in part of the cell’s protein quality control system, called the endoplasmic reticulum (ER), suggesting this may contribute to development of the disease.
This new mouse model will help unravel the molecular mechanisms of brain motor neuron vulnerability and may be useful for testing compounds to improve brain motor neuron health.
New Research Breakthrough
A small group of elusive neurons in the brain’s cortex play a big role in ALS, but the neurons have always been difficult to study because there are so few of them and they look so similar to other neurons in the cortex. In a new preclinical study, Dr. P. Hande Ozdinler isolated the motor neurons in the brain that die in ALS and, for the first time, dressed them in a green fluorescent jacket. Now they’re impossible to miss and easy to study.