Your Good Partner in Biology Research

  1. Home
  2. 抗体
  3. 多克隆抗体
  4. ISCU Antibody

ISCU Antibody

  • 货号：
    CSB-PA887955ESR2HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Immunohistochemistry of paraffin-embedded human lung cancer using CSB-PA887955ESR2HU at dilution of 1:100
    • Immunohistochemistry of paraffin-embedded human endometrial cancer using CSB-PA887955ESR2HU at dilution of 1:100
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) ISCU Polyclonal antibody
  • Uniprot No.：
    Q9H1K1
  • 基因名：
    ISCU
  • 别名：
    2310020H20Rik antibody; HML antibody; hnifU antibody; Iron sulfur cluster assembly enzyme ISCU mitochondrial antibody; Iron sulfur cluster scaffold homolog (E. coli) antibody; Iron sulfur cluster scaffold homolog antibody; Iron-sulfur cluster assembly enzyme ISCU antibody; Iscu antibody; IscU iron sulfur cluster scaffold homolog antibody; ISCU_HUMAN antibody; ISU2 antibody; MGC74517 antibody; mitochondrial antibody; NIFU antibody; NifU like N terminal domain containing antibody; NifU like N terminal domain containing protein antibody; NifU like protein antibody; NifU-like N-terminal domain-containing protein antibody; NifU-like protein antibody; NIFUN antibody; Nitrogen fixation cluster like antibody; OTTHUMP00000238760 antibody; OTTHUMP00000238761 antibody; OTTHUMP00000238762 antibody; OTTHUMP00000238764 antibody; OTTHUMP00000238765 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Iron-sulfur cluster assembly enzyme ISCU, mitochondrial protein (1-167AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity Purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:20-1:200
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

产品评价

靶点详情

  • 功能：
    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 Functions as a cytoplasmic scaffold protein for the de novo synthesis of iron-sulfur clusters in the cytoplasm.
  • 基因功能参考文献：
    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

    显示更多

    收起更多

  • 相关疾病：
    Myopathy with exercise intolerance Swedish type (MEIS)
  • 亚细胞定位：
    [Isoform 1]: Mitochondrion.; [Isoform 2]: Cytoplasm. Nucleus.
  • 蛋白家族：
    NifU family
  • 组织特异性：
    Detected in heart, liver, skeletal muscle, brain, pancreas, kidney, lung and placenta.
  • 数据库链接：

    HGNC: 29882

    OMIM: 255125

    KEGG: hsa:23479

    STRING: 9606.ENSP00000310623

    UniGene: Hs.615131