Product Description
Recombinant Human Iron-sulfur cluster assembly enzyme ISCU, mitochondrial (ISCU) is available at Gentaur for Next week Delivery.
Gene Name: ISCU
Alternative Names : NifU-like N-terminal domain-containing protein NifU-like protein
Expression Region : 1-167aa
AA Sequence : MAAAGAFRLRRAASALLLRSPRLPARELSAPARLYHKKVVDHYENPRNVGSLDKTSKNVGTGLVGAPACGDVMKLQIQVDEKGKIVDARFKTFGCGSAIASSSLATEWVKGKTVEEALTIKNTDIAKELCLPPVKLHCSMLAEDAIKAALADYKLKQEPKKGEAEKK
Sequence Info : Full Length
Tag Info : N-terminal GST-tagged
Theoretical MW : 45.0 kDa
Storage Buffer : Tris/PBS-based buffer, 5%-50% glycerol. If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.
Endotoxin Level : Not tested-
Biological Activity : Not tested
Storage : Short term: -20°C; Long term: -80°C. Minimize freeze and thaw cycles.
Research Area : Signal Transduction
Restriction : For Research Use Only. Not for use in diagnostic procedures, drug use, or for administration to humans or animals.
Relevance : 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
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
Involvement in disease : Myopathy with exercise intolerance Swedish type (MEIS)
Subcellular location : Isoform 1: Mitochondrion, SUBCELLULAR LOCATION: Isoform 2: Cytoplasm, Nucleus
Protein Families : NifU family
Tissue Specificity : Detected in heart, liver, skeletal muscle, brain, pancreas, kidney, lung and placenta.
Paythway :
Uniprot ID : Q9H1K1