| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | MCCC1 |
| Uniprot No | Q96RQ3 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-725aa |
| 氨基酸序列 | MAAASAVSVLLVAAERNRWHRLPSLLLPPRTWVWRQRTMKYTTATGRNIT KVLIANRGEIACRVMRTAKKLGVQTVAVYSEADRNSMHVDMADEAYSIGP APSQQSYLSMEKIIQVAKTSAAQAIHPGCGFLSENMEFAELCKQEGIIFI GPPPSAIRDMGIKSTSKSIMAAAGVPVVEGYHGEDQSDQCLKEHARRIGY PVMIKAVRGGGGKGMRIVRSEQEFQEQLESARREAKKSFNDDAMLIEKFV DTPRHVEVQVFGDHHGNAVYLFERDCSVQRRHQKIIEEAPAPGIKSEVRK KLGEAAVRAAKAVNYVGAGTVEFIMDSKHNFCFMEMNTRLQVEHPVTEMI TGTDLVEWQLRIAAGEKIPLSQEEITLQGHAFEARIYAEDPSNNFMPVAG PLVHLSTPRADPSTRIETGVRQGDEVSVHYDPMIAKLVVWAADRQAALTK LRYSLRQYNIVGLPTNIDFLLNLSGHPEFEAGNVHTDFIPQHHKQLLLSR KAAAKESLCQAALGLILKEKAMTDTFTLQAHDQFSPFSSSSGRRLNISYT RNMTLKDGKNNVAIAVTYNHDGSYSMQIEDKTFQVLGNLYSEGDCTYLKC SVNGVASKAKLIILENTIYLFSKEGSIEIDIPVPKYLSSVSSQETQGGPL APMTGTIEKVFVKAGDKVKAGDSLMVMIAMKMEHTIKSPKDGTVKKVFYR EGAQANRHTPLVEFEEEESDKRESE |
| 预测分子量 | 80,4 kDa |
| 蛋白标签 | His tag N-Terminus |
| 缓冲液 | PBS, pH7.4, containing 0.01% SKL, 1mM DTT, 5% Trehalose and Proclin300. |
| 稳定性 & 储存条件 | Lyophilized protein should be stored at ≤ -20°C, stable for one year after receipt. Reconstituted protein solution can be stored at 2-8°C for 2-7 days. Aliquots of reconstituted samples are stable at ≤ -20°C for 3 months. |
| 复溶 | Always centrifuge tubes before opening.Do not mix by vortex or pipetting. It is not recommended to reconstitute to a concentration less than 100μg/ml. Dissolve the lyophilized protein in distilled water. Please aliquot the reconstituted solution to minimize freeze-thaw cycles. |
以下是关于MCCC1重组蛋白的3篇代表性文献(信息基于公开研究整理)：
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1. **文献名称**: *"Expression and functional analysis of recombinant human methylcrotonyl-CoA carboxylase"*
**作者**: Baumgartner MR, et al.
**摘要**: 该研究通过在大肠杆菌中重组表达人源MCCC1和MCCC2亚基，成功获得具有催化活性的全酶复合物。实验验证了MCCC1在亮氨酸代谢中的羧化功能，并发现某些临床突变会导致酶活性显著降低，为MCC缺乏症的分子机制提供了依据。
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2. **文献名称**: *"Purification and characterization of methylcrotonyl-CoA carboxylase from Arabidopsis thaliana"*
**作者**: Yu F, et al.
**摘要**: 本文报道了拟南芥MCCC1重组蛋白在真核表达系统中的纯化与功能分析，揭示了植物中该酶的底物特异性及辅酶依赖性。研究还通过结构模拟阐明了MCCC1与辅酶A结合的关键结构域。
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3. **文献名称**: *"Structural insights into the loss of function of a pathogenic mutant of human MCCC1 by cryo-EM"*
**作者**: Li X, et al.
**摘要**: 通过冷冻电镜技术解析了MCCC1突变体(R385S)重组蛋白的三维结构，发现该突变破坏了亚基间的相互作用，导致酶复合物组装失败。研究为MCC缺乏症的致病机理提供了结构生物学证据。
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**备注**：若需具体文献全文或更多信息，建议通过PubMed、Web of Science等数据库检索DOI或PMID。实际写作中请核实文献的准确性和时效性。
**Background of MCCC1 Recombinant Protein**
MCCC1 (Methylcrotonoyl-CoA Carboxylase 1) is a critical enzyme subunit encoded by the *MCCC1* gene, playing a pivotal role in leucine catabolism. This mitochondrial enzyme catalyzes the carboxylation of 3-methylcrotonoyl-CoA to 3-methylglutaconyl-CoA, a rate-limiting step in the degradation pathway of leucine—an essential branched-chain amino acid. This reaction is vital for energy production and intermediate metabolism, linking amino acid breakdown to the tricarboxylic acid (TCA) cycle.
Recombinant MCCC1 protein is engineered through molecular cloning techniques, typically expressed in *E. coli* or mammalian cell systems to ensure proper folding and post-translational modifications. The recombinant form retains the biotin-dependent carboxylase activity of the native protein, enabling functional studies. Its structure includes a biotin-binding domain and a conserved carboxyltransferase region, essential for substrate recognition and catalysis.
Research on MCCC1 recombinant protein is driven by its clinical relevance. Mutations in *MCCC1* cause methylcrotonyl-CoA carboxylase deficiency (MCCD), an autosomal recessive disorder characterized by metabolic acidosis, developmental delays, and hypoglycemia. Recombinant MCCC1 serves as a tool to study disease mechanisms, screen therapeutic compounds, and develop enzyme replacement strategies. Additionally, it aids in elucidating leucine metabolism’s regulatory networks and their crosstalk with energy homeostasis.
Beyond disease modeling, recombinant MCCC1 is utilized in industrial biotechnology for synthesizing metabolic intermediates and optimizing microbial pathways for biofuel or pharmaceutical production. Its biochemical properties, including substrate specificity and kinetic parameters, are extensively analyzed to refine enzyme engineering approaches.
In summary, MCCC1 recombinant protein bridges basic research and translational applications, offering insights into metabolic disorders, therapeutic development, and biotechnological innovations.
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