| 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 | Glycine cleavage system H protein, mitochondrial, Lipoic acid-containing protein, GCSH |
| Entrez GeneID | 2653 |
| WB Predicted band size | 18.9kDa |
| 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 GCSH antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 27-56 amino acids from the N-terminal region of human GCSH. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide. |
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以下是关于GCSH (N-term)抗体的模拟参考文献示例(注：以下为构造示例，实际文献可能需要通过数据库进一步检索验证)：
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1. **文献名称**: "Immunological characterization of the N-terminal domain of GCSH in mitochondrial glycine metabolism"
**作者**: Zhang L, et al.
**摘要**: 本研究利用针对GCSH蛋白N端区域的多克隆抗体，通过Western blot和免疫荧光技术，揭示了GCSH在线粒体中的表达模式及其在甘氨酸裂解系统中的功能关联。实验验证了抗体在人类肝细胞系中的特异性。
2. **文献名称**: "Development of a monoclonal antibody targeting GCSH N-terminus for diagnostic applications"
**作者**: Tanaka K, et al.
**摘要**: 报道了一种新型GCSH N端单克隆抗体的开发，该抗体通过ELISA和免疫组化验证，能够特异性识别病理组织中的GCSH蛋白，为遗传性甘氨酸代谢紊乱的临床诊断提供了工具。
3. **文献名称**: "GCSH deficiency and its role in metabolic encephalopathy: Insights from antibody-based profiling"
**作者**: Müller R, et al.
**摘要**: 通过N端特异性抗体分析GCSH在患者脑组织中的表达水平，发现GCSH缺失与新生儿非酮症性高甘氨酸血症(NKH)相关，强调了其在神经代谢中的关键作用。
4. **文献名称**: "Structural insights into GCSH protein dynamics using epitope-specific antibodies"
**作者**: Wang Q, et al.
**摘要**: 利用针对GCSH不同结构域(包括N端)的抗体，结合X射线晶体学，解析了GCSH的构象变化，揭示了其与甘氨酸裂解复合体其他组分的相互作用机制。
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如需真实文献，建议通过PubMed或Google Scholar检索关键词“GCSH antibody N-terminal”或联系抗体供应商获取引用文献。
The GCSH (N-term) antibody is a specific immunological tool designed to target the N-terminal region of the glycine cleavage system H protein (GCSH), a critical component of the mitochondrial glycine cleavage system (GCS). This multienzyme complex, composed of four subunits (P, H, T, L), is essential for glycine catabolism, facilitating the conversion of glycine into carbon dioxide, ammonia, and a one-carbon unit for biosynthesis. GCSH, the H-protein, acts as a lipoamide-containing carrier that shuttles intermediates between the P- and T-proteins during glycine decarboxylation.
Antibodies targeting the N-terminal region of GCSH are widely used in research to study the expression, localization, and function of this protein in cellular and disease contexts. They are particularly valuable in diagnosing or investigating glycine encephalopathy (non-ketotic hyperglycinemia), a rare autosomal recessive disorder caused by mutations in GCS components, including GCSH. By enabling techniques like Western blotting, immunohistochemistry, and immunofluorescence, these antibodies help elucidate tissue-specific expression patterns, mitochondrial dysfunction mechanisms, and potential therapeutic targets.
Commercial GCSH (N-term) antibodies are typically raised in rabbits or mice using synthetic peptides corresponding to the N-terminal sequence of human GCSH. Their specificity is validated through knockout controls or siRNA-mediated knockdown. Researchers rely on these antibodies to explore metabolic disorders, cancer metabolism (where glycine metabolism may be dysregulated), and mitochondrial pathologies, contributing to advancements in molecular diagnostics and targeted therapies.
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