MDA has awarded a research grant totaling $316,557 over a period of three years to Brian Kraemer, a research biologist in the Geriatrics Research Education and Clinical Center at the Veterans Affairs Puget Sound Health Care System in Seattle.
The funds will help support Kraemer's study of the connection between mutated TDP43 protein and motor neuron (nerve cell) degeneration in amyotrophic lateral sclerosis (ALS).
Abnormal TDP43 protein has been observed in the affected brain and spinal cord nerve cells in people with ALS, and mutations in the gene for TDP43 have been shown to cause inherited ALS in some families.
The way in which abnormalities in the TDP43 protein cause nerve cells to die in ALS remains unclear, Kraemer said, noting, however, that phosphorylation, or the “addition of [chemicals called] phosphates, to TDP43 at abnormal positions is the most constant hallmark of ALS-related nerve cell destruction seen in post-mortem examination of the nervous systems of affected patients.”
Kraemer and colleagues plan to identify the proteins responsible for TDP43 phosphorylation. Then, in a mouse model of ALS, the investigators will test whether inhibition of the abnormal addition of phosphates to TDP43 could be a valid neuroprotective strategy for ALS.