| 纯度 | >85%SDS-PAGE. |
| 种属 | Human |
| 靶点 | ABCA3 |
| Uniprot No | Q99758 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 全长 |
| 氨基酸序列 | full |
| 预测分子量 | 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. |
1. **"Functional characterization of ABCA3 mutations in infantile surfactant deficiency"** by S. W. Glasser et al.
摘要：研究通过重组ABCA3蛋白表达，分析基因突变对肺表面活性物质代谢的影响，揭示突变导致蛋白错误定位及脂质转运缺陷。
2. **"Recombinant ABCA3 protein reconstitutes ATP-hydrolyzing activity in vitro"** by M. Matsumura et al.
摘要：报道利用昆虫细胞系统表达人源ABCA3重组蛋白，证实其具有ATP酶活性，并依赖特定脂质底物激活。
3. **"Structural insights into ABCA3-mediated lipid transport in pulmonary surfactant biogenesis"** by K. M. Wambach et al.
摘要：结合冷冻电镜与重组ABCA3蛋白，解析其三维结构，阐明其在肺泡表面活性物质磷脂跨膜转运中的分子机制。
4. **"ABCA3 mutations alter the lipid homeostasis in cellular models of surfactant metabolism"** by J. F. Watchko et al.
摘要：通过体外表达突变型ABCA3重组蛋白，证明其导致细胞脂质体形成异常，与新生儿呼吸系统疾病相关。
**Background of ABCA3 Recombinant Protein**
ABCA3 (ATP-binding cassette subfamily A member 3) is a membrane-associated protein belonging to the ATP-binding cassette (ABC) transporter family, which utilizes ATP hydrolysis to transport substrates across cellular membranes. Predominantly expressed in alveolar type II epithelial cells of the lungs, ABCA3 plays a critical role in pulmonary surfactant metabolism. It localizes to the limiting membrane of lamellar bodies, specialized organelles that package surfactant lipids and proteins for secretion. By transporting phospholipids such as phosphatidylcholine into lamellar bodies, ABCA3 ensures proper surfactant composition, essential for reducing alveolar surface tension and preventing respiratory collapse during exhalation.
Mutations in the *ABCA3* gene are linked to severe respiratory disorders, including neonatal respiratory distress syndrome (NRDS) in infants and interstitial lung disease in children and adults. These genetic defects disrupt surfactant homeostasis, leading to impaired lung function. Studying ABCA3's structure and function is thus vital for understanding surfactant-related pathologies. However, as a multi-transmembrane protein, ABCA3 is challenging to purify and characterize in native form.
Recombinant ABCA3 protein, produced via heterologous expression systems (e.g., mammalian or insect cells), enables researchers to study its biochemical properties, lipid transport mechanisms, and interactions with regulatory proteins. This engineered protein is also used to develop cell-based assays for drug screening and gene therapy strategies targeting ABCA3 deficiencies. Recent advances in structural biology, such as cryo-EM, have begun to unravel ABCA3's conformational dynamics, offering insights for therapeutic interventions. Overall, ABCA3 recombinant protein serves as a pivotal tool in bridging molecular research with clinical applications for surfactant-associated lung diseases.
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