The fact that ubiquilins interact also with proteins involved in spinocerebellar ataxia type 1 (UBQLN4 with ataxin-1;[9,50]] and Huntington’s disease (UBLQN1 with huntingtin [51]), that UBLQN1 and UBQLN2 proteins are found in protein aggregates in Huntington disease models and human brains affected by Huntington and other neurodegenerative diseases [52-54] and the finding of UBQLN1 mutations in Brown-Vialetto-Van Laere syndrome, a rare neurological disease [55], further suggested important roles of these proteins in neural tissues. This evidence concerns the gene UBQLN4 and juvenile Huntington disease.