Recombinant Human Iron-sulfur cluster assembly enzyme ISCU, mitochondrial(ISCU)

N-terminal GST-tagged

45.0 kDa

Scaffold protein for the de novo synthesis of iron-sulfur (Fe-S) clusters within mitochondria, which is required for maturation of both mitochondrial and cytoplasmic [2Fe-2S] and [4Fe-4S] proteins. First, a [2Fe-2S] cluster is transiently assembled on the scaffold protein ISCU. In a second step, the cluster is released from ISCU, transferred to a glutaredoxin GLRX5, followed by the formation of mitochondrial [2Fe-2S] proteins, the synthesis of [4Fe-4S] clusters and their target-specific insertion into the recipient apoproteins. Cluster assembly on ISCU depends on the function of the cysteine desulfurase complex NFS1-LYRM4/ISD11, which serves as the sulfur donor for cluster synthesis, the iron-binding protein frataxin as the putative iron donor, and the electron transfer chain comprised of ferredoxin reductase and ferredoxin, which receive their electrons from NADH

Repeated freezing and thawing is not recommended. Store working aliquots at 4C for up to one week.

Isoform 1: Mitochondrion, SUBCELLULAR LOCATION: Isoform 2: Cytoplasm, Nucleus

Myopathy with exercise intolerance Swedish type (MEIS)

Full Length

Tris-based buffer, 50% glycerol