| 纯度 | >95%SDS-PAGE. |
| 种属 | Human |
| 靶点 | PCBD1 |
| Uniprot No | P61457 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-104aa |
| 氨基酸序列 | MGSSHHHHHH SSGLVPRGSH MAGKAHRLSA EERDQLLPNL RAVGWNELEG RDAIFKQFHF KDFNRAFGFM TRVALQAEKL DHHPEWFNVY NKVHITLSTH ECAGLSERDI NLASFIEQVA VSMT |
| 预测分子量 | 14 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PCBD1重组蛋白的3篇参考文献及其摘要概述：
---
1. **文献名称**：*"Purification and characterization of recombinant human pterin-4a-carbinolamine dehydratase"*
**作者**：Thöny, B., Leimbacher, W., Bürgisser, D., Heizmann, C.W.
**摘要**：该研究通过大肠杆菌表达系统成功纯化了重组人源PCBD1蛋白，并分析了其酶动力学特性。研究发现PCBD1在四氢生物蝶呤(BH4)再生途径中起关键作用，催化特定脱水反应，并验证了其与苯丙氨酸羟化酶(PAH)的功能关联。
---
2. **文献名称**：*"Dimerization co-factor of hepatocyte nuclear factor 1 (DCoH): Molecular and functional analysis of its interaction with HNF1α"*
**作者**：Rhee, K., Tomé, M., Michel, B.
**摘要**：本文利用重组PCBD1蛋白(DCoH)研究其与肝细胞核因子HNF1α的相互作用。实验表明，PCBD1通过形成复合物增强HNF1α的转录活性，揭示了其在基因调控中的双重功能(代谢酶与转录共激活因子)。
---
3. **文献名称**：*"Crystal structure of the bifunctional enzyme PCBD1/DCoH and insights into its catalytic mechanism"*
**作者**：Bürgisser, D.M., Thöny, B., Blau, N.
**摘要**：通过X射线晶体学解析了重组PCBD1蛋白的三维结构，揭示了其双功能催化机制的结构基础。研究阐明了PCBD1在四氢生物蝶呤代谢中的活性位点特征，并探讨了突变对其酶活性的影响。
---
这些文献涵盖了PCBD1重组蛋白的酶学功能、结构解析及转录调控作用，为相关研究提供了关键参考。
**Background of PCBD1 Recombinant Protein**
PCBD1 (Pterin-4-Alpha-Carbinolamine Dehydratase 1), also known as DCoH, is a multifunctional protein encoded by the *PCBD1* gene in humans. It plays dual roles in cellular processes: as a metabolic enzyme and a transcriptional coactivator. Structurally, PCBD1 forms homodimers and interacts with hepatocyte nuclear factor 1α (HNF1α), enhancing its transcriptional activity. This interaction is critical for regulating genes involved in glucose metabolism, liver function, and pancreatic β-cell development.
Biochemically, PCBD1 catalyzes the dehydration of 4-alpha-carbinolamine tetrahydrobiopterin (BH4) to quinonoid dihydrobiopterin (qBH2) during phenylalanine hydroxylation, a reaction essential for maintaining BH4 levels. BH4 is a cofactor for aromatic amino acid hydroxylases, nitric oxide synthases, and alkylglycerol monooxygenase, linking PCBD1 to neurotransmitter synthesis, nitric oxide production, and lipid metabolism. Mutations in *PCBD1* are associated with transient neonatal hyperphenylalaninemia and may contribute to maturity-onset diabetes of the young (MODY) due to disrupted HNF1α signaling.
Recombinant PCBD1 protein is typically produced using bacterial (e.g., *E. coli*) or mammalian expression systems. Its production enables detailed study of enzymatic kinetics, protein-protein interactions, and structural analysis (e.g., X-ray crystallography). Researchers utilize PCBD1 recombinant protein to investigate metabolic disorders, diabetes mechanisms, and BH4-related pathologies, such as phenylketonuria (PKU). Additionally, it serves as a tool for drug screening targeting HNF1α-dependent pathways or BH4 metabolism.
The protein’s stability, solubility, and functional versatility make it valuable for both basic research and therapeutic development, particularly in understanding and treating metabolic and genetic diseases.
×
