Table 1. Primarily Affected Tissues in Fe−S Cluster Synthesis Diseasesa.
| FXN | GLRX5 | ISCU | ABCB7 | |
|---|---|---|---|---|
| erythroblasts | + | + | ||
| sensory and cerebellar neurons | + | + | ||
| cardiomyocytes | + | + | ||
| skeletal myocytes | + |
The primarily affected cell types are sensory neurons and cardiac myocytes for frataxin-deficient Friedreich's ataxia, erythroblasts and cerebellar neurons for ABCB7-deficient XLSA/A, skeletal and cardiac myocytes for ISCU-deficient myopathy, and erythroblasts for GLRX5-deficient sideroblastic anemia.