High levels of PEG10 have been implicated in the neurological diseases Angelman’s syndrome [28] and ALS [17, 38], where regulators of PEG10 abundance (UBE3A in Angelman’s, and UBQLN2 in ALS) are mutated and unable to control PEG10 levels. This evidence concerns the gene UBQLN2 and amyotrophic lateral sclerosis.