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Recombinant Human PCCb protein

  • 中文名: 丙酰辅酶A羧化酶β(PCCb)重组蛋白
  • 别    名: PCCb;Propionyl-CoA carboxylase beta chain, mitochondrial
货号: PA2000-901DB
Price: ¥询价
数量:
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产品详情

纯度>90%SDS-PAGE.
种属Human
靶点PCCb
Uniprot NoP05166
内毒素< 0.01EU/μg
表达宿主E.coli
表达区间7-266aa
氨基酸序列VAAVGARLSVLASGLRAAVRSLCSQATSVNERIENKRRTALLGGGQRRIDAQHKRGKLTARERISLLLDPGSFVESDMFVEHRCADFGMAADKNKFPGDSVVTGRGRINGRLVYVFSQDFTVFGGSLSGAHAQKICKIMDQAITVGAPVIGLNDSGGARIQEGVESLAGYADIFLRNVTASGVIPQISLIMGPCAGGAVYSPALTDFTFMVKDTSYLFIT
预测分子量28 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.

参考文献

以下是关于PCCb(假设为丙酰辅酶A羧化酶β亚基)重组蛋白研究的示例性参考文献,仅供参考。实际文献需通过学术数据库检索获取:

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1. **《重组人丙酰辅酶A羧化酶β亚基的表达与功能分析》**

- 作者:Zhang Y, et al.

- 摘要:研究利用大肠杆菌系统成功表达并纯化重组人PCCb蛋白,证实其具有体外酶活性,为酶缺陷相关疾病(如丙酸血症)的治疗提供实验基础。

2. **《丙酰辅酶A羧化酶β亚基的结构解析与重组表达优化》**

- 作者:Johnson R, et al.

- 摘要:通过基因工程技术优化PCCb的重组表达条件,结合X射线晶体学解析其三维结构,揭示关键催化位点,助力靶向药物设计。

3. **《基于哺乳动物细胞的重组PCCb蛋白在代谢疾病模型中的应用》**

- 作者:Lee S, et al.

- 摘要:在HEK293细胞中高效表达功能性重组PCCb蛋白,验证其在丙酸血症小鼠模型中改善代谢异常的效果,推动酶替代疗法的临床前研究。

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**提示**:实际研究中建议以“Propionyl-CoA carboxylase beta subunit recombinant”为关键词,在PubMed、Google Scholar等平台检索,并筛选近年高相关性文献。

背景信息

**Background of PCCβ Recombinant Protein**

PCCβ (Propionyl-CoA Carboxylase beta subunit) is a critical enzyme subunit involved in the mitochondrial catabolism of branched-chain amino acids, cholesterol, and fatty acids. It forms a heterodimeric complex with the alpha subunit (PCCα) to constitute propionyl-CoA carboxylase (PCC), a biotin-dependent enzyme essential for propionate metabolism. PCC catalyzes the carboxylation of propionyl-CoA to methylmalonyl-CoA, a key step in the tricarboxylic acid (TCA) cycle. Defects in PCCβ, encoded by the *PCCB* gene, are linked to propionic acidemia, an autosomal recessive metabolic disorder characterized by life-threatening acidosis and multi-organ dysfunction.

Recombinant PCCβ protein is engineered to study its structure-function relationships, disease mechanisms, and therapeutic potential. Produced via heterologous expression systems (e.g., *E. coli*, mammalian cells), it retains enzymatic activity when assembled with PCCα, enabling in vitro analysis of PCC function. Researchers utilize recombinant PCCβ to investigate mutations causing propionic acidemia, screen small-molecule chaperones to restore enzyme activity, and develop enzyme replacement therapies (ERT) or gene therapies.

Recent advances in protein engineering and structural biology (e.g., cryo-EM) have elucidated PCCβ's role in substrate binding and biotin coordination, aiding drug design. Recombinant PCCβ also serves as a diagnostic antigen or reference material in clinical assays. Despite challenges in stabilizing the large, multi-domain protein, ongoing studies aim to optimize its production and delivery for translational applications, offering hope for targeted treatments for propionic acidemia and related metabolic disorders.

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