Faculty Opinions recommendation of Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6.

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Abstract
This manuscript is important because it provides a likely molecular mechanism for amyotrophic lateral sclerosis (ALS). ALS is a serious degenerative disease that has familial origin in about 10% of all cases, and studies on familial cases of this illness have allowed the authors to identify a candidate set of mutations in the gene coding for the FUS protein. Interestingly, this factor is an RNA-processing protein, and the mutations appear to alter the subcellular location of this protein and cause the formation of cytoplasmic inclusions. While the relationships between these molecular events and neurodegeneration remains inconclusive, this manuscript (and an accompanying manuscript {1}) represents a significant step forward in understanding this illness.

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