| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | PHKA2 |
| Uniprot No | P46019 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-1235 aa |
| 活性数据 | MRSRSNSGVRLDGYARLVQQTILCYQNPVTGLLSASHEQKDAWVRDNIYSILAVWGLGMAYRKNADRDEDKAKAYELEQNVVKLMRGLLQCMMRQVAKVEKFKHTQSTKDSLHAKYNTATCGTVVGDDQWGHLQVDATSLFLLFLAQMTASGLRIIFTLDEVAFIQNLVFYIEAAYKVADYGMWERGDKTNQGIPELNASSVGMAKAALEAIDELDLFGAHGGRKSVIHVLPDEVEHCQSILFSMLPRASTSKEIDAGLLSIISFPAFAVEDVNLVNVTKNEIISKLQGRYGCCRFLRDGYKTPREDPNRLHYDPAELKLFENIECEWPVFWTYFIIDGVFSGDAVQVQEYREALEGILIRGKNGIRLVPELYAVPPNKVDEEYKNPHTVDRVPMGKVPHLWGQSLYILSSLLAEGFLAAGEIDPLNRRFSTSVKPDVVVQVTVLAENNHIKDLLRKHGVNVQSIADIHPIQVQPGRILSHIYAKLGRNKNMNLSGRPYRHIGVLGTSKLYVIRNQIFTFTPQFTDEHHFYLALDNEMIVEMLRIELAYLCTCWRMTGRPTLTFPISRTMLTNDGSDIHSAVLSTIRKLEDGYFGGARVKLGNLSEFLTTSFYTYLTFLDPDCDEKLFDNASEGTFSPDSDSDLVGYLEDTCNQESQDELDHYINHLLQSTSLRSYLPPLCKNTEDRHVFSAIHSTRDILSVMAKAKGLEVPFVPMTLPTKVLSAHRKSLNLVDSPQPLLEKVPESDFQWPRDDHSDVDCEKLVEQLKDCSNLQDQADILYILYVIKGPSWDTNLSGQHGVTVQNLLGELYGKAGLNQEWGLIRYISGLLRKKVEVLAEACTDLLSHQKQLTVGLPPEPREKIISAPLPPEELTKLIYEASGQDISIAVLTQEIVVYLAMYVRAQPSLFVEMLRLRIGLIIQVMATELARSLNCSGEEASESLMNLSPFDMKNLLHHILSGKEFGVERSVRPIHSSTSSPTISIHEVGHTGVTKTERSGINRLRSEMKQMTRRFSADEQFFSVGQAASSSAHSSKSARSSTPSSPTGTSSSDSGGHHIGWGERQGQWLRRRRLDGAINRVPVGFYQRVWKILQKCHGLSIDGYVLPSSTTREMTPHEIKFAVHVESVLNRVPQPEYRQLLVEAIMVLTLLSDTEMTSIGGIIHVDQIVQMASQLFLQDQVSIGAMDTLEKDQATGICHFFYDSAPSGAYGTMTYLTRAVASYLQELLPNSGCQMQ |
| 分子量 | 161.59 kDa |
| 蛋白标签 | GST-tag at N-terminal |
| 缓冲液 | 0 |
| 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. |
以下是关于重组人PHKA2蛋白的3篇代表性文献示例(注：文献为模拟示例，实际引用请核实)：
1. **文献名称**：*Expression and Functional Characterization of Recombinant Human PHKA2 in a Glycogen Storage Disease Model*
**作者**：Chen L, et al.
**摘要**：本研究通过杆状病毒-昆虫细胞系统成功表达了重组人PHKA2蛋白，并验证了其与α亚基结合形成磷酸化酶激酶复合物的能力。实验表明，PHKA2的缺陷导致激酶活性丧失，揭示了其在IXa型糖原贮积症中的分子机制。
2. **文献名称**：*Structural Insights into PHKA2 Mutations Disrupting Phosphorylase Kinase Activation*
**作者**：Wang Y, et al.
**摘要**：利用X射线晶体学解析了重组PHKA2蛋白的结构，发现其C端结构域对激酶激活至关重要。文章分析了临床突变体R50H和L87R对蛋白构象的影响，为疾病诊断提供了结构基础。
3. **文献名称**：*Recombinant PHKA2 Production in HEK293 Cells: Implications for Enzyme Replacement Therapy*
**作者**：Smith J, et al.
**摘要**：开发了HEK293细胞高效表达重组PHKA2的方法，纯化后蛋白可恢复患者源性细胞中磷酸化酶激酶活性，为IXa型糖原贮积症的潜在治疗策略提供了实验依据。
(注：以上文献信息为示例性质，实际研究中请通过PubMed或学术数据库核实具体文献。)
Phosphorylase kinase α2 (PHKA2) is a regulatory subunit of the phosphorylase kinase (PhK) complex, a key enzyme in glycogen metabolism. This hexadecameric enzyme complex, primarily expressed in the liver and muscle, regulates glycogen breakdown by phosphorylating and activating glycogen phosphorylase. PHKA2 contains two major domains: a calmodulin-binding regulatory region and a catalytic α-subunit interaction domain. It plays dual roles in both sensing calcium signals (via calmodulin-like motifs) and maintaining PhK structural integrity.
Mutations in the PHKA2 gene cause X-linked liver glycogen storage disease type IX (GSD IX), characterized by hepatomegaly, growth delays, and intermittent hypoglycemia. Over 200 variants are documented, with phenotype severity correlating to residual enzyme activity. Recombinant human PHKA2 protein production typically employs mammalian expression systems to ensure proper post-translational modifications. This engineered protein enables biochemical studies of PhK assembly, structure-function relationships, and mutation impacts. It also serves as a critical reagent for developing targeted therapies and diagnostic tools for GSD IX. Recent research explores PHKA2's emerging roles beyond glycogenolysis, including potential involvement in insulin signaling and cellular stress responses.
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