纯度 | >90%SDS-PAGE. |
种属 | Human |
靶点 | PANK2 |
Uniprot No | Q9BZ23 |
内毒素 | < 0.01EU/μg |
表达宿主 | E.coli |
表达区间 | 47-570aa |
氨基酸序列 | SLSL DGGTVNPPRV REPTGREAFG PSPASSDWLP ARWRNGRGGR PRARLCSGWT AAEEARRNPT LGGLLGRQRL LLRMGGGRLG APMERHGRAS ATSVSSAGEQ AAGDPEGRRQ EPLRRRASSA SVPAVGASAE GTRRDRLGSY SGPTSVSRQR VESLRKKRPL FPWFGLDIGG TLVKLVYFEP KDITAEEEEE EVESLKSIRK YLTSNVAYGS TGIRDVHLEL KDLTLCGRKG NLHFIRFPTH DMPAFIQMGR DKNFSSLHTV FCATGGGAYK FEQDFLTIGD LQLCKLDELD CLIKGILYID SVGFNGRSQC YYFENPADSE KCQKLPFDLK NPYPLLLVNI GSGVSILAVY SKDNYKRVTG TSLGGGTFFG LCCLLTGCTT FEEALEMASR GDSTKVDKLV RDIYGGDYER FGLPGWAVAS SFGNMMSKEK REAVSKEDLA RATLITITNN IGSIARMCAL NENINQVVFV GNFLRINTIA MRLLAYALDY WSKGQLKALF SEHEGYFGAV GALLELLKIP |
预测分子量 | 62,6 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. |
以下是3条关于PANK2重组蛋白的参考文献摘要概括:
1. **"Functional characterization of human PANK2 mutations reveals substrate-dependent enzyme kinetics"** - Johnson et al.
研究通过重组表达人源PANK2蛋白,发现PKAN相关突变导致酶对ATP和泛酸的亲和力显著降低,揭示了突变体催化活性受损的分子机制。
2. **"Crystal structure of pantothenate kinase 2 reveals conformational flexibility in substrate binding"** - Zhang et al.
利用X射线晶体学解析了重组PANK2蛋白的三维结构,发现其ATP结合域存在动态构象变化,为设计靶向药物提供了结构基础。
3. **"Recombinant PANK2 as a tool for high-throughput drug screening in neurodegeneration"** - Vanni et al.
开发了基于重组PANK2蛋白的体外酶活检测体系,筛选出多个可恢复突变体活性的小分子化合物,具有治疗PKAN的潜力。
注:以上为虚拟文献概括,实际研究中建议通过PubMed检索关键词“PANK2 recombinant”“pantothenate kinase 2 expression”获取真实文献(如涉及PANK2重组表达与功能研究的核心论文多发表于《Biochimica et Biophysica Acta》《Human Molecular Genetics》等期刊)。
**Background of PANK2 Recombinant Protein**
PANK2 (pantothenate kinase 2) is a key enzyme in coenzyme A (CoA) biosynthesis, catalyzing the phosphorylation of pantothenate (vitamin B5) to initiate the CoA synthesis pathway. This mitochondrial enzyme is encoded by the *PANK2* gene, mutations in which are linked to neurodegeneration with brain iron accumulation (NBIA), specifically pantothenate kinase-associated neurodegeneration (PKAN). PKAN is a rare autosomal recessive disorder characterized by dystonia, parkinsonism, and iron deposition in the basal ganglia, highlighting PANK2's critical role in cellular metabolism and neuronal health.
Recombinant PANK2 protein is produced using expression systems (e.g., *E. coli* or mammalian cells) to study its biochemical properties, structure-function relationships, and disease mechanisms. Its production enables in vitro analysis of enzyme kinetics, substrate specificity, and the impact of PKAN-associated mutations (e.g., missense variants), which often destabilize the protein or reduce catalytic activity. Structural studies reveal that PANK2 forms homodimers with distinct regulatory and catalytic domains, and its dysfunction disrupts CoA homeostasis, leading to metabolic stress and neurodegeneration.
Research on recombinant PANK2 also supports therapeutic development, including small-molecule activators, gene therapy, or enzyme replacement strategies. Additionally, it serves as a tool for screening compounds to correct folding defects or enhance residual enzyme activity in PKAN models. Despite progress, challenges remain in understanding how PANK2 variants drive iron accumulation and neuronal death, underscoring the need for further mechanistic studies using recombinant protein-based approaches.
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