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| Reactivity: | H,M,R |
| Applications: | WB |
| Host Species: | Rabbit |
| Clonality: | Polyclonal |
| Gene Name: | Iron-sulfur cluster assembly enzyme ISCU, mitochondrial |
Synonyms: | 2310020H20Rik, HML, hnifU, ISCU, ISCU2, ISU2, NIFU, NifU like protein, NIFUN |
Immunogen: | Recombinant protein corresponding to Human ISCU |
Uniprot ID: | Q9H1K1 |
Isotype: | IgG |
Purity: | Affinity purification |
Predicted MW. / Observed MW. | 18 kDa / 14 kDa |
Product Usage Information
WB | Human, Mouse, Rat | 1: 500-1: 1000 | liver, pancreas |
Background
This gene encodes a component of the iron-sulfur (Fe-S) cluster scaffold. Fe-S clusters are cofactors that play a role in the function of a diverse set of enzymes, including those that regulate metabolism, iron homeostasis, and oxidative stress response. Alternative splicing results in transcript variants encoding different protein isoforms that localize either to the cytosol or to the mitochondrion. Mutations in this gene have been found in patients with hereditary myopathy with lactic acidosis. A disease-associated mutation in an intron may activate a cryptic splice site, resulting in the production of a splice variant encoding a putatively non-functional protein. A pseudogene of this gene is present on chromosome 1.
Images
| Western blot analysis of ISCU (GB114213) at dilution of 1: 1000 |
Storage
| Storage | Store at -20°C for one year. Avoid repeated freeze/thaw cycles. |
| Storage Buffer | PBS with 0.02%sodium azide,100 μg/ml BSA and 50% glycerol. |
