Fabry's disease: Heterozygote detection by hair root analysis

Abstract
Fabry's disease (angiokeratoma corporis diffusum) can be diagnosed by determination of α-galactosidase activity in single hair roots. A technique has been developed permitting subsequent analysis of enzyme activity and protein content of hair root cells. We have applied this method examining 8 obligatorily heterozygous sisters as well as their 5 daughters and 10 sons. Especially for identification of heterozygotes, the method described proved to be easy and reliable.