Washington: Scientists claim to have discovered the common cause of all forms of amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disease that paralyses its victims.The underlying disease process of ALS has long eluded doctors and prevented development of effective therapies.Now, a team at Northwestern University has for the first time discovered that the basis of the disorder is a broken down protein -- identified as ubiquilin2 -- recycling system in the neurons of the spinal cord and the brain.Optimal functioning of the neurons relies on efficient recycling of the protein building blocks in the cells. In ALS, that recycling system is broken. The cell can`t repair or maintain itself and becomes severely damaged.In fact, ubiquilin2`s critical job is to recycle damaged or misfolded proteins in motor and cortical neurons and shuttle them off to be reprocessed. In people with ALS, it isn`t doing its job, the scientists found.As a result, the damaged proteins and ubiquilin2 loiter and accumulate in the motor neurons in the spinal cord and cortical and hippocampal neurons in the brain. The protein accumulations resemble twisted skeins of yarn and cause the degeneration of the neurons.The discovery, published in the journal Nature, provides a common target for drug therapy and shows that all types of ALS are, indeed, tributaries, pouring into a common river of cellular incompetence.
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