| 產(chǎn)品編號 | bs-17865R |
| 英文名稱 | MT-ATP6 Rabbit pAb |
| 中文名稱 | ATP6蛋白抗體 |
| 別 名 | ATP synthase subunit a; ATP6; ATP6_HUMAN; ATPASE6; F-ATPase protein 6; MT-ATP6; MTATP6 |
| 研究領(lǐng)域 | 心血管 細胞生物 免疫學(xué) 神經(jīng)生物學(xué) 表觀遺傳學(xué) |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 克 隆 號 | |
| 交叉反應(yīng) | Human |
| 產(chǎn)品應(yīng)用 | IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 25 kDa |
| 檢測分子量 | |
| 細胞定位 | 細胞核 細胞膜 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Msx3: 21-120/226 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase or Complex V) produces ATP from ADP in the presence of a proton gradient across the membrane which is generated by electron transport complexes of the respiratory chain. F-type ATPases consist of two structural domains, F(1) - containing the extramembraneous catalytic core and F(0) - containing the membrane proton channel, linked together by a central stalk and a peripheral stalk. During catalysis, ATP synthesis in the catalytic domain of F(1) is coupled via a rotary mechanism of the central stalk subunits to proton translocation. Key component of the proton channel; it may play a direct role in the translocation of protons across the membrane. Subcellular Location: Mitochondrion inner membrane. DISEASE: Defects in MT-ATP6 are the cause of neurogenic muscle weakness, ataxia, and retinitis pigmentosa (NARP) [MIM:551500]. Defects in MT-ATP6 are a cause of Leber hereditary optic neuropathy (LHON) [MIM:535000]. LHON is a maternally inherited disease resulting in acute or subacute loss of central vision, due to optic nerve dysfunction. Cardiac conduction defects and neurological defects have also been described in some patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes. Defects in MT-ATP6 are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions. Defects in MT-ATP6 are a cause of mitochondrial infantile bilateral striatal necrosis (MIBSN) [MIM:500003]. Bilateral striatal necrosis is a neurological disorder resembling Leigh syndrome. Similarity: Belongs to the ATPase A chain family. Gene ID: 4508 Database links:
Entrez Gene: 4508 Human Omim: 516060 Human |
| 產(chǎn)品圖片 |
Paraformaldehyde-fixed, paraffin embedded Human Cerebrum; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with MT-ATP6 Polyclonal Antibody, Unconjugated (bs-17865R) at 1:200 overnight at 4°C, followed by conjugation to the SP Kit (Rabbit, SP-0023) and DAB (C-0010) staining.
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