NDUFS4 Recombinant Monoclonal Antibody

說明書

中文名稱：

NDUFS4重組抗體
貨號：

CSB-RA588190A0HU
規格：

￥1320
圖片：
IHC image of CSB-RA588190A0HU diluted at 1:100 and staining in paraffin-embedded human small intestine tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB.

Overlay Peak curve showing MCF7 cells stained with CSB-RA588190A0HU (red line) at 1:100. The cells were fixed in 4% formaldehyde and permeated by 0.2% TritonX-100. Then 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (1ug/1*10⁶cells) for 45min at 4℃. The secondary antibody used was FITC-conjugated Goat Anti-rabbit IgG(H+L) at 1:200 dilution for 35min at 4℃.Control antibody (green line) was rabbit IgG (1ug/1*10⁶cells) used under the same conditions. Acquisition of >10,000 events was performed.
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品描述：

CSB-RA588190A0HU是靶向NDUFS4蛋白的重組單克隆抗體，該蛋白作為線粒體復合物I（NADH脫氫酶復合體）的核心亞基，參與氧化磷酸化與ATP生成過程，其功能異常與線粒體能量代謝紊亂及神經退行性疾病相關。本抗體通過ELISA、免疫組化（IHC）和流式細胞術（FC）嚴格驗證，展現出優異的特異性和重復性，推薦使用稀釋度為IHC 1:50-1:200、FC 1:50-1:200，適用于多種樣本類型檢測。在科研場景中，該抗體可高效應用于線粒體復合物I的功能研究、細胞能量代謝機制解析以及病理模型中NDUFS4蛋白的定位與表達水平檢測，為神經退行性疾病、代謝綜合征等領域的體外實驗提供可靠工具。其重組單克隆特性確保批次間一致性，滿足高通量實驗需求。
Uniprot No.：

O43181
基因名：

NDUFS4
別名：

NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial (Complex I-18 kDa) (CI-18 kDa) (Complex I-AQDQ) (CI-AQDQ) (NADH-ubiquinone oxidoreductase 18 kDa subunit), NDUFS4
反應種屬：

Human
免疫原：

A synthesized peptide derived from human NDUFS4
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
克隆類型：

Monoclonal
抗體亞型：

Rabbit IgG
純化方式：

Affinity-chromatography
克隆號：

9H1
濃度：

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

Rabbit IgG in 10mM phosphate buffered saline , pH 7.4, 150mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.
產品提供形式：

Liquid
應用范圍：

ELISA, IHC, FC

推薦稀釋比：

Application	Recommended Dilution
IHC	1:50-1:200
FC	1:50-1:200

Protocols：

ELISA Protocol
Immunohistochemistry (IHC) Protocol
Flow cytometry (FC) 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：

Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.
基因功能參考文獻：
1. The clinical presentations of five individuals of Hutterite descent with Leigh disease are described herein. An identity-by-descent mapping and candidate gene approach was used to identify a novel homozygous c.393dupA frameshift mutation in the NADH dehydrogenase (ubiquinone) Fe-S protein 4 (NDUFS4) gene. PMID: 27671926
2. The authors concluded that NDUFS4-related Leigh syndrome is invariably linked to an early onset severe phenotype that results in early death. PMID: 27079373
3. The c.462delA deletion led to a complete lack of NDUFS4 peptide in isolated mitochondria, and this deficiency caused an inefficient mitochondrial complex I assembly and Leigh syndrome symptoms. PMID: 24020637
4. Mutations in the NDUFS4 gene and its subunits are associated with the mitochondrial complex I deficiency. (Review) PMID: 23378164
5. Elevated expression of CO I and ND4 were associated with gastric tumorigenesis and tumor dedifferentiation PMID: 22407105
6. Studies indicate that that the functional capacity of complex I depends on phosphorylation and import of subunit NDUFS4 protein. PMID: 21945319
7. In fibroblast cultures, protein kinase A-mediated phosphorylation of the NDUFS4 subunit of complex I rescues the activity of the oxidatively damaged complex. PMID: 22198267
8. case Report: A novel mutation in NDUFS4 causes Leigh syndrome in an Ashkenazi Jewish family. PMID: 19107570
9. REVIEW: Phosphorylation of the NDUFS4 protein, overall respiratory activity, and mutations sassociated with deficiency of complex I. PMID: 11860175
10. In patients with complex I deficiency, the increased whole-body oxygen consumption rate at rest reflects increased electron transport through the respiratory chain, driven by a decreased phosphorylation potential. PMID: 11940698
11. observations show the essential role of the 18-kDa subunit of respiratory complex I (NDUFS4) gene in the structure and function of complex I and give insight into pathogenic mechanism of NDUFS4 gene mutations in a severe defect of complex I PMID: 12944388
12. A nonsense mutation leading to the abrogation of mRNA decay was found in NDUFS4 gene of a Leigh syndrome patient. PMID: 15975579
13. NDUSF4 is required for the assembly and stabilization of a portion of complex I that contains a number of subunits. PMID: 17438127
14. impact of PKA mediated phosphorylation on the mitochondrial import of in vitro and in vivo synthesized NDUFS4 protein PMID: 18291624
15. regulation of alternative transcripts of the NDUFS4 gene of complex I of the respiratory chain PMID: 18555024
16. NDUFS4 presents a hotspot of mutations in the genetic apparatus of oxidative phosphorylation and the correct assembly of the subunit it encodes is essential for completion of the assembly of complex I. PMID: 19364667
顯示更多

收起更多
相關疾病：

Mitochondrial complex I deficiency (MT-C1D); Leigh syndrome (LS)
亞細胞定位：

Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
蛋白家族：

Complex I NDUFS4 subunit family
數據庫鏈接：

HGNC: 7711

OMIM: 252010

KEGG: hsa:4724

STRING: 9606.ENSP00000296684

UniGene: Hs.528222

Most popular with customers

YWHAG Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC

Species Reactivity: Human
YWHAB Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC, IF, FC

Species Reactivity: Human, Mouse, Rat
Phospho-YAP1 (S127) Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC

Species Reactivity: Human
XRCC5 Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC, IF

Species Reactivity: Human
XPNPEP1 Recombinant Monoclonal Antibody

Applications: ELISA, IHC, FC

Species Reactivity: Human
XDH Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC

Species Reactivity: Human
VIM Recombinant Monoclonal Antibody

Applications: ELISA, IHC, IF

Species Reactivity: Human
VDR Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC

Species Reactivity: Human