| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | DNAJC19 |
| Uniprot No | Q96DA6 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 19-116aa |
| 氨基酸序列 | MRGSHHHHHH GMASMTGGQQ MGRDLYDDDD KDRWGSMGRY VLQAMKHMEP QVKQVFQSLP KSAFSGGYYR GGFEPKMTKR EAALILGVSP TANKGKIRDA HRRIMLLNHP DKGGSPYIAA KINEAKDLLE GQAKK |
| 预测分子量 | 15 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DNAJC19重组蛋白的3篇代表性文献摘要，供参考：
1. **文献名称**: *DNAJC19. a mitochondrial cochaperone, interacts with mitochondrial HSP70 and is essential for early embryonic development*
**作者**: Davey KM等
**摘要**: 本研究利用重组DNAJC19蛋白验证其与线粒体HSP70的相互作用，揭示其在维持线粒体蛋白折叠中的分子伴侣功能，基因敲除小鼠模型显示DNAJC19缺失导致胚胎致死，提示其对线粒体稳态的关键调控作用。
2. **文献名称**: *Mutations in DNAJC19 disrupt mitochondrial iron homeostasis and cause cardiomyopathy with anemia*
**作者**: Baertling F等
**摘要**: 通过表达重组突变型DNAJC19蛋白，研究者发现其突变(如p.R145W)会损害线粒体铁硫簇合成，导致患者出现扩张型心肌病和铁粒幼细胞性贫血，揭示了DNAJC19在铁代谢中的新功能。
3. **文献名称**: *DNAJC19 interacts with prohibitins and regulates cristae morphology in mitochondria*
**作者**: Richter-Dennerlein R等
**摘要**: 利用重组DNAJC19与PHB复合体的体外结合实验，证实其通过调控线粒体内膜超微结构维持嵴形态，缺失DNAJC19导致线粒体呼吸链复合体组装异常，影响能量代谢效率。
注：实际文献检索建议通过PubMed/Google Scholar以"DNAJC19 recombinant"+"mitochondria"等关键词获取最新研究。部分研究可能侧重于疾病关联而非重组蛋白直接分析。
**Background of DNAJC19 Recombinant Protein**
DNAJC19 is a member of the evolutionarily conserved J-protein family (Hsp40/DNAJ family), which functions as a co-chaperone for heat shock protein 70 (HSP70) in regulating protein folding, trafficking, and degradation. It is primarily localized to the mitochondrial inner membrane and plays a critical role in mitochondrial dynamics, energy metabolism, and stress responses. DNAJC19 interacts with the TIM23 complex, facilitating the import of nuclear-encoded proteins into mitochondria.
Mutations in the *DNAJC19* gene are linked to a rare autosomal recessive disorder called 3-methylglutaconic aciduria type V (MGCA5), characterized by dilated cardiomyopathy, ataxia, and growth retardation. This association underscores its importance in mitochondrial integrity and cellular homeostasis.
Recombinant DNAJC19 protein is engineered through molecular cloning, typically expressed in *E. coli* or mammalian systems, to produce purified, functional protein for research. Its recombinant form enables studies on molecular mechanisms underlying mitochondrial dysfunction, protein interaction networks, and disease pathology. Researchers utilize it to investigate DNAJC19’s role in HSP70-mediated folding, its structural domains (e.g., the J-domain responsible for HSP70 binding), and its involvement in metabolic pathways.
Additionally, recombinant DNAJC19 serves as a tool for screening therapeutic compounds targeting mitochondrial disorders or cardiomyopathies. Understanding its structure-function relationship aids in elucidating how mutations disrupt mitochondrial protein import or chaperone activity, offering insights into potential therapeutic strategies for MGCA5 and related conditions.
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