AR and bone Paget disease: ALS: amyotrophic lateral sclerosis, SOD: superoxide dismutase, SETX: senataxin, dHMN; distal hereditary motor neuropathies, ANG: angiogenin, PMA: progressive muscular atrophy, FTD: frontotemporal dementia, PBP: progressive bulbar palsy, OPTN: optineurin, TARDBP: transactive response DNA binding protein, ATXN2: ataxin-2, VAPB: VAMP Associated Protein B and C, FIG: Fused in Glioblastoma, PLS: Primary Lateral Sclerosis, SPG: spastic paraplegia, VCP: Valocin Containing Protein, Arg487: Arginase487, PDB: Paget's disease of bone, UBQLN2: ubiquilin-2, AD: autosomal dominant, AR: autosomal recessive