Iron-sulfur (Fe-S) clusters are necessary for several
mitochondrial enzymes and other subcellular compartment proteins.
They contain sulfur and iron, and are created via several steps
that include cysteine desulfurases, iron donors, chaperones, and
scaffold proteins. This gene encodes the two isomeric forms, ISCU1
and ISCU2, of the Fe-S cluster scaffold protein. Mutations in this
gene have been found in patients with myopathy with severe exercise
intolerance and myoglobinuria.
