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

Code CSB-EP887955HU(A4)
Size US$1726
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  • (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.

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Product Details

Purity Greater than 90% as determined by SDS-PAGE.
Target Names ISCU
Uniprot No. Q9H1K1
Research Area Signal Transduction
Alternative Names 2310020H20Rik; HML; hnifU; Iron sulfur cluster assembly enzyme ISCU mitochondrial; Iron sulfur cluster scaffold homolog (E. coli); Iron sulfur cluster scaffold homolog; Iron-sulfur cluster assembly enzyme ISCU; Iscu; IscU iron sulfur cluster scaffold homolog; ISCU_HUMAN; ISU2; MGC74517; mitochondrial; NIFU; NifU like N terminal domain containing; NifU like N terminal domain containing protein; NifU like protein; NifU-like N-terminal domain-containing protein; NifU-like protein; NIFUN; Nitrogen fixation cluster like; OTTHUMP00000238760; OTTHUMP00000238761; OTTHUMP00000238762; OTTHUMP00000238764; OTTHUMP00000238765
Species Homo sapiens (Human)
Source E.coli
Expression Region 1-167aa
Target Protein Sequence MAAAGAFRLRRAASALLLRSPRLPARELSAPARLYHKKVVDHYENPRNVGSLDKTSKNVGTGLVGAPACGDVMKLQIQVDEKGKIVDARFKTFGCGSAIASSSLATEWVKGKTVEEALTIKNTDIAKELCLPPVKLHCSMLAEDAIKAALADYKLKQEPKKGEAEKK
Note: The complete sequence including tag sequence, target protein sequence and linker sequence could be provided upon request.
Mol. Weight 45.0kDa
Protein Length Full Length
Tag Info N-terminal GST-tagged
Form Liquid or Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
Buffer If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol.
Note: If you have any special requirement for the glycerol content, please remark when you place the order.
If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.
Reconstitution We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20°C/-80°C. Our default final concentration of glycerol is 50%. Customers could use it as reference.
Troubleshooting
and FAQs
Protein FAQs
Storage Condition Store at -20°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
Shelf Life The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
Lead Time Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Notes Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet & COA Please contact us to get it.

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Target Data

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
Gene References into Functions
  1. identifies an important regulatory role for zinc-bound ISCU in modulation of the human Fe-S assembly system in vitro and reports no 'FXN bypass' effect on mutations at position Met140 in human ISCU PMID: 30031876
  2. we report the first heterozygous dominant mutation in ISCU; notably, this alteration resulted in a similar phenotype as the recessive ISCU disease previously described. PMID: 29079705
  3. When ISCU was replaced by the fully structured variant ISCU(M108I), the addition of rdFDX2 to the [NIA-ISCU(M108I)-FXN]2 complex led to the release of FXN. Thus, the displacement of FXN by rdFDX2 explains the failure of FXN to stimulate Fe-S cluster assembly on ISCU(M108I). PMID: 29406711
  4. We have shown that ASO treatment diminished aberrant splicing and increased ISCU protein levels in both patient fibroblasts and patient myotubes in a concentration dependent fashion. Upon ASO treatment, levels of SDHB in patient myotubular cell lines increased to levels observed in control myotubular cell lines PMID: 28007899
  5. The NFS1/ISD11 complex further interacts with scaffold protein ISCU and regulator protein frataxin, thereby forming a quaternary complex for Fe-S cluster formation. PMID: 28271877
  6. Molecular dynamics flexible fitting of protein structures docked into the EM map of the model revealed a [FXN(42-210)]24.[NFS1]24.[ISD11]24.[ISCU]24 complex, consistent with the measured 1:1:1:1 stoichiometry of its four components. PMID: 27519411
  7. ISCU expression was decreased in the majority of human liver cancer tissues, and its reduced expression was significantly associated with p53 mutation. PMID: 26560363
  8. Thus, driven by acquired (hypoxia) or genetic causes, the miR-210-ISCU1/2 regulatory axis is a pathogenic lynchpin causing iron-sulfur deficiency and pulmonary hypertension. PMID: 25825391
  9. The core Fe-S biosynthetic enzymatic complex generated [2Fe-2S] cluster intermediates that converted to stable [2Fe-2S] clusters bound to uncomplexed ISCU2. PMID: 26016389
  10. IscU is a new substrate of MK2 both in Drosophila cells and in human cells PMID: 25204651
  11. Fe-S assembly protein (ISCU2) and frataxin convert substrates l-cysteine, ferrous iron, and electrons into Fe-S clusters. PMID: 24971490
  12. the G50E iron-sulfur cluster scaffold protein (ISCU) mutation has a role in mitochondrial myopathy PMID: 24573684
  13. NFS1 binds preferentially to the D-state of ISCU while mtHSP70 binds preferentially to the D-state of ISCU and HSC20 binds preferentially to the S-state of ISCU. PMID: 23940031
  14. mTORC1 associates with ISCU and phosphorylates ISCU at serine 14. This phosphorylation stabilized ISCU protein. PMID: 23508953
  15. MicroRNA-210 correlates negatively with its gene target ISCU at the protein and mRNA level. MicroRNA-210 correlated with positive outcome variables PMID: 23449350
  16. ISCU protein deficiency in patients results from muscle-specific mis-splicing and oxidative stress. PMID: 23035118
  17. this study unveiled a signaling axis of HIF-1alpha/miRNA-210/iron-sulfur cluster scaffold protein in a subset of head and neck paragangliomas that could have an impact on SDHB protein stability by a mechanism independent of succinate dehydrogenase mutations PMID: 22977270
  18. Photoreactive heterotrifunctional chemical cross-linking confirmed the interaction between frataxin and ISCU in the presence of iron and validated that transient interactions can be covalently trapped with this method. PMID: 22897349
  19. iron-sulfur cluster scaffold homologue down-regulation by miR-210 perturbing trophoblast iron metabolism is associated with defective placentation PMID: 21801864
  20. Exchange of [2Fe-2S] centers between glutaredoxin 2 and the cluster scaffold protein ISU, supports a direct link for glutaredoxin 2 and glutathione involvement in ISU promoted Fe-S cluster biosynthesis. PMID: 21437321
  21. Data show that the highest level of incorrectly spliced ISCU mRNA was found in skeletal muscle. PMID: 21165651
  22. miR-210 mediates a new mechanism of adaptation to hypoxia, by regulating mitochondrial function via iron-sulfur cluster metabolism and free radical generation PMID: 20436681
  23. ISCU and COX10 are target genes of miR-210 related to mitochondrial metabolism PMID: 20498629
  24. Data suggest that frataxin may be involved in the biosynthesis of iron-sulphur proteins such as IscU1 not only within the mitochondria, but also in the extramitochondrial compartment. PMID: 16091420
  25. Functional analysis of the mitochondrial and cytosolic isoforms of the human Fe-S cluster scaffold protein, ISCU. PMID: 16517407
  26. the cytosolic form of ISCS is a functional cysteine desulfurase that can collaborate with cytosolic ISCU to promote de novo iron-sulfur cluster formation PMID: 16527810
  27. Intron mutation in the ISCU gene, leading to incorrectly spliced mRNA, is the cause of myopathy with lactic acidosis in this family. PMID: 18296749
  28. Gene ISCU was identified as a candidate within a region of shared homozygosity among patients with myopathy with severe exercise intolerance and myoglobinuria. PMID: 18304497
  29. the iron-dependent binding affinity of each frataxin derivative to the iron-sulfur cluster scaffold protein ISU is found to be similar to that of native frataxin PMID: 18425540
  30. a new ISCU mutation in iron-sulphur cluster deficiency myopathy PMID: 19567699
  31. Results identify the iron-sulfur cluster assembly proteins (ISCU1/2) as direct targets for repression by the hypoxia-induced microRNA-210. PMID: 19808020

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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.
Database Links

HGNC: 29882

OMIM: 255125

KEGG: hsa:23479

STRING: 9606.ENSP00000310623

UniGene: Hs.615131

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