CLDN17 and amyotrophic lateral sclerosis: The 2 Mbp region contained few notable protein-coding genes including GRIK1, CLDN8, and CLDN17, 15 genes for keratin-associated proteins, TIAM1, SOD1, and SCAF4. Of these, only mutations in SOD1 were known for well-established neurologic phenotypes in humans and dogs: amyotrophic lateral sclerosis type 1 (ALS1, OMIM 105400) and progressive spastic tetraplegia and axial hypotonia (OMIM 618598), and degenerative myelopathy (DM, OMIA 000263–9615), respectively.