Our results clearly link CSPα to the motor neuron disorder ALS and polyglutamine expansion disorders like Huntington’s disease and are congruent with a recent study by Fontaine and colleagues who reported that release of TDP-43, α-synuclein and microtubule-associated protein tau from HEK-293 cells is enhanced by CSPα39. This evidence concerns the gene TARDBP and juvenile Huntington disease.