| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 咨询技术 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | Surfeit locus protein 1; SURF1; SURF-1; |
| Entrez GeneID | 6834; |
| WB Predicted band size | 30kDa |
| Host/Isotype | Rabbit IgG |
| Antibody Type | Primary antibody |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human,Mouse,Rat |
| Immunogen | Synthesized peptide derived from internal of human SURF1. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide. |
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以下是3篇关于SURF1抗体的参考文献及其摘要概括：
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1. **标题**：Mutations of SURF-1 in Leigh disease associated with cytochrome c oxidase deficiency
**作者**：Tiranti, V. et al.
**摘要**：该研究首次发现SURF1基因突变导致Leigh综合征(亚急性坏死性脑脊髓病)及细胞色素c氧化酶(COX)缺陷。通过Western blot和免疫组化实验，使用SURF1抗体证实患者细胞中SURF1蛋白表达缺失，揭示SURF1在COX组装中的关键作用。
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2. **标题**：SURF1. encoding a factor involved in the biogenesis of cytochrome c oxidase, is mutated in Leigh syndrome
**作者**：Zhu, Z. et al.
**摘要**：研究克隆了SURF1基因并开发了特异性抗体，用于分析患者成纤维细胞中的蛋白表达。结果显示SURF1突变导致蛋白截短或降解，且与线粒体复合物IV(COX)功能受损直接相关，为Leigh综合征的分子机制提供依据。
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3. **标题**：The role of the SURF1 gene in the assembly of cytochrome c oxidase
**作者**：Antonicka, H. et al.
**摘要**：通过免疫沉淀和蛋白质组学分析，结合SURF1抗体，研究发现SURF1蛋白直接参与COX的早期组装步骤。实验表明，SURF1缺失导致COX亚基稳定性下降，强调其在维持线粒体呼吸链完整性中的功能。
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4. **标题**：Diagnostic utility of SURF1 antibody in mitochondrial disorders
**作者**：Hanna, M.G. et al.
**摘要**：评估SURF1抗体在临床诊断中的应用，通过免疫荧光和Western blot检测患者样本，证实抗体可有效区分SURF1相关线粒体病与其他类型COX缺陷，为快速诊断提供可靠工具。
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以上文献均涉及SURF1抗体的实验应用，涵盖疾病机制、蛋白功能及临床诊断方向。
The SURF1 (Surfeit locus protein 1) antibody is a research tool used to study the SURF1 protein, a critical factor in mitochondrial function. SURF1 is localized in the inner mitochondrial membrane and plays a key role in the assembly of mitochondrial complex IV (cytochrome c oxidase), a component of the electron transport chain essential for cellular energy production. Mutations in the SURF1 gene are associated with Leigh syndrome, a severe neurodegenerative disorder characterized by progressive loss of motor skills and mitochondrial respiratory chain defects.
SURF1 antibodies are primarily utilized in biomedical research to detect and quantify SURF1 protein expression in tissues or cultured cells. These antibodies enable techniques such as Western blotting, immunohistochemistry, and immunofluorescence, helping researchers investigate SURF1's role in mitochondrial biogenesis, disease mechanisms, and potential therapeutic strategies. Commercial SURF1 antibodies are typically raised in hosts like rabbits or mice, targeting specific epitopes of the human SURF1 protein. Validation often includes testing in SURF1-deficient models to confirm specificity.
Studies employing SURF1 antibodies have advanced understanding of mitochondrial disorders, particularly how SURF1 dysfunction disrupts complex IV assembly, leading to bioenergetic failure. Ongoing research focuses on correlating SURF1 expression patterns with disease severity and exploring gene therapy approaches.
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