X-linked vacuolar myopathies can be divided into two forms: one that is ass
ociated with cardiomyopathy and mental retardation (XVCM-MR) and a second f
orm, termed X-linked myopathy with excessive autophagy (XMEA), that spares
cardiac muscle and has no central nervous system involvement. In this artic
le, we demonstrate linkage between XMEA and markers on chromosome Xq28 and
assign the XMEA gene locus to the most telomeric 10.5 cM of chromosome X. W
e also show that XVCM-MR is not allelic to XMEA.