ISCU Antibody, Biotin conjugated

說明書

中文名稱：

ISCU兔多克隆抗體, Biotin偶聯
貨號：

CSB-PA887955DD01HU
規格：

￥880
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) ISCU Polyclonal antibody
Uniprot No.：

Q9H1K1
基因名：

ISCU
別名：

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
反應種屬：

Human
免疫原：

Recombinant Human Iron-sulfur cluster assembly enzyme ISCU, mitochondrial protein (35-167AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

Biotin
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
產品提供形式：

Liquid
應用范圍：

ELISA
Protocols：

ELISA Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

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.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

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.
基因功能參考文獻：
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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相關疾?。?/div>
Myopathy with exercise intolerance Swedish type (MEIS)
亞細胞定位：

[Isoform 1]: Mitochondrion.; [Isoform 2]: Cytoplasm. Nucleus.
蛋白家族：

NifU family
組織特異性：

Detected in heart, liver, skeletal muscle, brain, pancreas, kidney, lung and placenta.
數據庫鏈接：

HGNC: 29882

OMIM: 255125

KEGG: hsa:23479

STRING: 9606.ENSP00000310623

UniGene: Hs.615131

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