WB | 1/1000 | 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 | Collagen alpha-3(IV) chain, Goodpasture antigen, Tumstatin, COL4A3 |
Entrez GeneID | 1285 |
WB Predicted band size | 161.8kDa |
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 |
Immunogen | This COL4A3 antibody is generated from a rabbit immunized with a KLH conjugated synthetic peptide between 141-173 amino acids from human COL4A3. |
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以下是关于COL4A3 (N-Term)抗体的3篇代表性文献示例(注:文献信息为示例性概括,非真实存在):
1. **文献名称**:*"Localization of COL4A3 in Glomerular Basement Membranes Using N-Terminal Antibodies"*
**作者**:Smith A, et al.
**摘要**:通过针对COL4A3蛋白N端的特异性抗体,研究揭示了该蛋白在肾小球基底膜中的分布模式,并证实其在Alport综合征患者中的表达缺失。
2. **文献名称**:*"Autoantibodies Targeting COL4A3-N-Terminal Epitopes in Anti-GBM Disease"*
**作者**:Zhang L, et al.
**摘要**:研究发现,抗肾小球基底膜(GBM)疾病患者体内存在靶向COL4A3 N端结构域的自身抗体,提示该区域可能是免疫反应的关键表位。
3. **文献名称**:*"COL4A3 Knockout Mouse Model and N-Terminal Antibody Validation"*
**作者**:Brown K, et al.
**摘要**:利用COL4A3 N端抗体验证了Col4a3基因敲除小鼠模型中基底膜结构的异常,为研究遗传性肾病提供了工具支持。
4. **文献名称**:*"Diagnostic Utility of COL4A3 Antibodies in Renal Biopsies"*
**作者**:Lee S, et al.
**摘要**:评估了COL4A3 N端抗体在肾脏病理诊断中的应用,显示其在鉴别Alport综合征与其他肾小球疾病中的高特异性。
(注:以上文献为示例,实际引用需检索真实数据库如PubMed。)
The COL4A3 (N-Term) antibody targets the N-terminal region of the alpha-3 chain of type IV collagen, a critical component of basement membranes. Type IV collagen forms a network essential for the structural integrity and selective filtration functions of basement membranes in tissues such as kidneys, cochlea, and eyes. The α3 chain, encoded by the COL4A3 gene, combines with α4 and α5 chains to create heterotrimers predominantly found in the glomerular basement membrane (GBM) and alveolar basement membranes.
Mutations in COL4A3 are linked to Alport syndrome, a genetic disorder characterized by progressive kidney disease, hearing loss, and ocular abnormalities. The N-terminal domain plays a role in collagen network assembly, and its dysfunction disrupts GBM structure, leading to proteinuria and renal failure. The COL4A3 (N-Term) antibody is widely used in research to study basement membrane defects, diagnose Alport syndrome, and investigate related conditions like thin basement membrane nephropathy. It aids in detecting COL4A3 expression via techniques such as immunohistochemistry, Western blotting, and immunofluorescence, providing insights into disease mechanisms and potential therapeutic targets. This antibody is crucial for understanding the molecular basis of collagen IV-associated pathologies and advancing diagnostic or treatment strategies.
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