In according with these findings, other neurological disorders of the upper motor neuron with onset in early childhood, including “intellectual disability”, motor dysfunction and joint contractures (IDMDC)” were also related to a deficiency expression of ERLIN2 [130,136], although it cannot be ruled out that ERLIN2 may exert its pathogenic role through the impaired degradation of other proteins; in fact, this mechanism remains to be investigated in ALS phenotype [137]. Here, ERLIN2 is linked to amyotrophic lateral sclerosis.