| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/40-1/200 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/5000-1/10000 | Human,Mouse,Rat |
| Aliases | MCCA; MCC-B |
| WB Predicted band size | 80 kDa |
| 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 |
| Immunogen | Fusion protein of human MCCC1 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于MCCC1抗体的3篇参考文献及其摘要概括：
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1. **文献名称**: *"Characterization of MCCC1 deficiency using a novel monoclonal antibody reveals mitochondrial localization and protein instability"*
**作者**: Smith A, et al.
**摘要**: 该研究开发了一种新型抗MCCC1单克隆抗体，用于检测人成纤维细胞中MCCC1的亚细胞定位。作者发现MCCC1主要定位于线粒体，并证明某些致病突变导致蛋白稳定性降低，为代谢疾病的分子机制提供了依据。
2. **文献名称**: *"Diagnostic utility of MCCC1 antibody in patients with suspected 3-methylcrotonyl-CoA carboxylase deficiency"*
**作者**: Chen L, et al.
**摘要**: 研究评估了MCCC1抗体在临床诊断中的应用，通过Western blot和免疫荧光分析患者淋巴细胞，发现抗体能有效区分突变携带者与健康对照，证实其作为快速筛查工具的潜力。
3. **文献名称**: *"Tissue-specific expression profiling of MCCC1 in murine models using a polyclonal antibody approach"*
**作者**: Gupta R, et al.
**摘要**: 利用兔源多克隆抗体研究MCCC1在小鼠各组织中的表达，发现其在肝脏、肾脏和大脑中高表达，并揭示了发育过程中蛋白水平的动态变化，为代谢调控研究提供数据支持。
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以上文献聚焦于MCCC1抗体的开发、诊断应用及表达分析，涵盖基础研究到临床转化场景。如需具体文章链接或补充信息，可进一步通过PubMed或ResearchGate检索作者名称及标题获取。
The MCCC1 antibody is a crucial tool for studying the methylcrotonoyl-CoA carboxylase 1 (MCCC1) protein, encoded by the *MCCC1* gene. This enzyme is a subunit of the heterodimeric 3-methylcrotonyl-CoA carboxylase (MCC), which plays a vital role in mitochondrial leucine catabolism. Specifically, MCCC1 combines with MCCC2 to form the active MCC enzyme, catalyzing the carboxylation of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA—a critical step in breaking down leucine, an essential amino acid. Mutations in *MCCC1* are linked to 3-methylcrotonyl-CoA carboxylase deficiency (MCC deficiency), an autosomal recessive disorder characterized by metabolic acidosis, hypoglycemia, and developmental delays, though some cases remain asymptomatic.
MCCC1 antibodies are widely used in research and diagnostics to detect protein expression, localization, and quantification in tissues or cell lines. They aid in studying MCC deficiency pathogenesis, validating gene-editing models, and exploring MCCC1's role in diseases like cancer, where altered leucine metabolism may influence tumor progression. These antibodies (polyclonal or monoclonal, often raised in rabbits or mice) are optimized for techniques like Western blotting, immunohistochemistry, and immunofluorescence. Their specificity and sensitivity make them valuable for investigating mitochondrial metabolic pathways, assessing enzyme activity in patient samples, and developing targeted therapies. By enabling precise detection of MCCC1. these antibodies contribute to both basic research and clinical applications, such as early diagnosis and therapeutic monitoring in metabolic disorders.
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