|Other Names||Iron-sulfur cluster assembly enzyme ISCU, mitochondrial, NifU-like N-terminal domain-containing protein, NifU-like protein, ISCU, NIFUN|
|Target/Specificity||The synthetic peptide sequence is selected from aa 153-167 of HUMAN ISCU|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||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 (PubMed:11060020). 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 (By similarity).|
|Cellular Location||Isoform 1: Mitochondrion|
|Tissue Location||Detected in heart, liver, skeletal muscle, brain, pancreas, kidney, lung and placenta|
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Provided below are standard protocols that you may find useful for product applications.
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.
Chen, Z., et al. Oncogene 29(30):4362-4368(2010)
Chan, S.Y., et al. Cell Metab. 10(4):273-284(2009)
Kollberg, G., et al. Brain 132 (PT 8), 2170-2179 (2009) :
Huang, J., et al. J. Biol. Inorg. Chem. 13(5):825-836(2008)
Mochel, F., et al. Am. J. Hum. Genet. 82(3):652-660(2008)
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