Recombinant Human BBOX1 protein

中文名： 2-氧代戊二酸双加氧酶(BBOX1)重组蛋白
别名： BBOX1；BBH；BBOX；；Gamma-butyrobetaine dioxygenase

货号: PA1000-5777

Price： ￥询价

20μg 50μg 100μg ＞100μg

数量：

大包装询价

产品详情

纯度	>85%SDS-PAGE.
种属	Human
靶点	BBOX1
Uniprot No	O75936
内毒素	< 0.01EU/μg
表达宿主	E.coli
表达区间	1-387aa
氨基酸序列	MACTIQKAEALDGAHLMQILWYDEEESLYPAVWLRDNCPCSDCYLDSAKARKLLVEALDVNIGIKGLIFDRKKVYITWPDEHYSEFQADWLKKRCFSKQARAKLQRELFFPECQYWGSELQLPTLDFEDVLRYDEHAYKWLSTLKKVGIVRLTGASDKPGEVSKLGKRMGFLYLTFYGHTWQVQDKIDANNVAYTTGKLSFHTDYPALHHPPGVQLLHCIKQTVTGGDSEIVDGFNVCQKLKKNNPQAFQILSSTFVDFTDIGVDYCDFSVQSKHKIIELDDKGQVVRINFNNATRDTIFDVPVERVQPFYAALKEFVDLMNSKESKFTFKMNPGDVITFDNWRLLHGRRSYEAGTEISRHLEGAYADWDVVMSRLRILRQRVENGN
预测分子量	71.6 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BBOX1重组蛋白的3篇代表性文献的简要信息(注：文献为示例性概括，非真实存在)：

1. **"Cloning, expression, and characterization of recombinant human BBOX1 in Escherichia coli"**

*作者：Martini A, et al. (2002)*

**摘要**：报道了人源BBOX1基因的克隆及在大肠杆菌中的重组表达，优化了蛋白可溶性和纯化条件，并通过酶活实验验证其催化β-丙氨酸转化为肉碱前体的功能。

2. **"Structural insights into the substrate specificity of BBOX1 enzyme"**

*作者：Stieger B, et al. (2005)*

**摘要**：通过重组BBOX1蛋白的晶体结构解析，揭示了其底物结合口袋的关键氨基酸残基，阐明了其对β-丙氨酸和赖氨酸的特异性识别机制。

3. **"Functional analysis of BBOX1 mutations associated with metabolic disorders"**

*作者：Lee S, et al. (2016)*

**摘要**：利用重组BBOX1蛋白进行体外酶活实验，发现某些遗传突变导致酶活性显著降低，提示其与原发性肉碱缺乏症的相关性。

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如需真实文献，建议通过PubMed或Google Scholar搜索关键词："BBOX1 recombinant protein expression" 或 "BBOX1 enzymatic characterization"。

背景信息

BBOX1 (γ-butyrobetaine hydroxylase) is a key enzyme in the biosynthesis of L-carnitine, a molecule essential for fatty acid metabolism and energy production. It catalyzes the final step in the carnitine synthesis pathway, converting γ-butyrobetaine to L-carnitine using molecular oxygen, iron, and ascorbate as cofactors. This mitochondrial enzyme is primarily expressed in the liver, kidneys, and testes, playing a critical role in maintaining cellular energy homeostasis by facilitating the transport of long-chain fatty acids into mitochondria for β-oxidation.

Recombinant BBOX1 protein is engineered through genetic cloning and expression systems, typically using E. coli, yeast, or mammalian cell lines. The recombinant form preserves the enzymatic activity of native BBOX1 while enabling standardized production for research and therapeutic applications. Its production involves codon optimization, vector design, and purification techniques (e.g., affinity chromatography) to ensure high purity and stability. Structural studies reveal a conserved 2-His-1-carboxylate facial triad in its active site, characteristic of Fe²⁺/α-ketoglutarate-dependent dioxygenases.

Research on recombinant BBOX1 focuses on metabolic disorders like primary carnitine deficiency, diabetes, and cardiovascular diseases. It serves as a tool to study carnitine metabolism dysregulation and screen potential enzyme modulators. Recent investigations also explore its role in cancer progression, as altered lipid metabolism in tumors may depend on carnitine shuttle activity. Pharmaceutical interest lies in developing BBOX1 inhibitors for metabolic syndrome management or activators for carnitine supplementation therapies. However, challenges remain in understanding its tissue-specific regulation and interactions with other metabolic pathways.