Iron-Sulfur Cluster Assembly Enzyme ISCU, Mitochondrial (ISCU) Antibody
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Description
ISCU Antibody is a Rabbit Polyclonal antibody against ISCU. 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.
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Product specifications
| Category | Primary Antibodies |
| Immunogen Target | Iron-Sulfur Cluster Assembly Enzyme ISCU, Mitochondrial (ISCU) |
| Host | Rabbit |
| Reactivity | Human, Mouse |
| Recommended Dilution | ELISA: 1 µg/ml, WB: 1/500 - 1/1000. Optimal dilutions/concentrations should be determined by the end user. |
| Clonality | Polyclonal |
| Conjugation | Unconjugated |
| Isotype | IgG |
| Purification | Purified by affinity chromatography. |
| Size 1 | 20 µl |
| Size 2 | 100 µl |
| Size 3 | 2 × 100 µl |
| Form | Liquid |
| Tested Applications | ELISA, WB |
| Buffer | PBS, pH 7.3, containing 0.02% sodium azide, 50% glycerol. |
| Availability | Shipped within 5-10 working days. |
| Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
| Dry Ice | No |
| UniProt ID | Q9H1K1 |
| Gene ID | 23479 |
| NCBI Accession | NP_998760.1 |
| Background | Antibody anti-ISCU |
| Status | RUO |
| Note | Concentration: > 0.2 mg/ml - |
Descripción
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ISCU antibody
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).
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Recombinant Human ISCU
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Iron-Sulfur Cluster Assembly Enzyme ISCU, Mitochondrial (ISCU) Antibody
ISCU Antibody is a Rabbit Polyclonal antibody against ISCU. 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.
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