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Patients become progressively weaker until muscle use is entirely lost.
The research report appears in this month's Journal of Neuropathology and Experimental Neurology. The team next plans to test PEDF in transgenic mouse models of the disease. In the study, researchers pinpointed PEDF for the first time in the spinal cord and skeletal muscle of humans, monkeys and rats. Prior to this, scientists thought the eyes were its prime location, in the pigmented layer of cells underlying the retina. Levels of a key enzyme typically made by healthy cells underscored the result.