Grant Details

Does Wolframin Possess A Motor Neuron Stabilizing Function?
Kottmann, PhD, Andreas
Columbia University, New York, NY

Project Summary
This investigator has discovered that a mutation responsible for a disease called the Wolfram Syndrome (WS) is at work in motor neurons and in a select subset of neurons in temporal-frontal brain areas. He demonstrated that the protein made by this gene, called wolframin, is in the receiving fibers of motor neurons. Kottmann has produced animals that lack the wfs1 gene, which begin to exhibit progressive motor problems at eight weeks of age and go on to show many features similar to ALS. The protein produced by the gene might normally help keep motor neurons healthy. The aim of the proposed work is to see if the wolframin protein, over-expressed within lab grown motor neurons, can protect these cells when challenged by the toxic action of excessive glutamate or by co-presence of the mutant SOD1 protein responsible for some inherited forms of ALS.

Related Research Topics
Laboratory Models of ALS