Absence of linkage between familial amyotrophic lateral sclerosis and copper chaperone for the superoxide dismutase gene locus in two Italian pedigrees

We investigated the segregation of the copper chaperone for the superoxide dismutase (CCS) gene in two Italian families with amyotrophic lateral scler osis lacking the mutations in superoxide dismutase 1 gene. We analyzed a to tal of 56 individuals; six people were affected. Diagnoses were made using the El Escorial criteria. The results of our study provide no evidence of a linkage between markers flanking the CCS gene and familial amyotrophic lat eral sclerosis (FALS) in these FALS kindreds. (C) 2000 Elsevier Science Ire land Ltd. All rights reserved.