Mutations in GBE1 can cause autosomal recessively inherited adult Polyglucosan body disease (APBD) which is characterized by upper motor neuron signs similar to ALS, early neurogenic bladder, cognitive impairment and decreased or absent activity of the glycogen branching enzyme (Klein, 2013). The gene discussed is GBE1; the disease is glycogen storage disease due to glycogen branching enzyme deficiency.