| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | ALS |
| Uniprot No | P35858 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-605aa |
| 氨基酸序列 | MALRKGGLALALLLLSWVALGPRSLEGADPGTPGEAEGPACPAACVCSYD DDADELSVFCSSRNLTRLPDGVPGGTQALWLDGNNLSSVPPAAFQNLSSL GFLNLQGGQLGSLEPQALLGLENLCHLHLERNQLRSLALGTFAHTPALAS LGLSNNRLSRLEDGLFEGLGSLWDLNLGWNSLAVLPDAAFRGLGSLRELV LAGNRLAYLQPALFSGLAELRELDLSRNALRAIKANVFVQLPRLQKLYLD RNLIAAVAPGAFLGLKALRWLDLSHNRVAGLLEDTFPGLLGLRVLRLSHN AIASLRPRTFKDLHFLEELQLGHNRIRQLAERSFEGLGQLEVLTLDHNQL QEVKAGAFLGLTNMAVMNLSGNCLRNLPEQVFRGLGKLHSLHLEGSCLGR IRPHTFTGLSGLRRLFLKDNGLVGIEEQSLWGLAELLELDLTSNQLTHLP HRLFQGLGKLEYLLLSRNRLAELPADALGPLQRAFWLDVSHNRLEALPNS LLAPLGRLRYLSLRNNSLRTFTPQPPGLERLWLEGNPWDCGCPLKALRDF ALQNPSAVPRFVQAICEGDDCQPPAYTYNNITCASPPEVVGLDLRDLSEA HFAPC |
| 预测分子量 | 93 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篇与ALS(肌萎缩侧索硬化症)重组蛋白研究相关的文献概览：
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1. **文献名称**：*Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis*
**作者**：Rosen, D.R., Siddique, T., Patterson, D. 等
**摘要**：该研究首次发现SOD1基因突变与家族性ALS的关联，通过重组蛋白技术表达突变型SOD1.揭示了其异常折叠和聚集导致运动神经元退化的机制。
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2. **文献名称**：*TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis*
**作者**：Sreedharan, J., Blair, I.P., Tripathi, V.B. 等
**摘要**：利用重组TDP-43蛋白研究其在ALS中的作用，发现突变导致蛋白错误定位和细胞毒性，揭示了RNA代谢异常在ALS病理中的关键作用。
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3. **文献名称**：*Dysregulation of autophagy as a common mechanism in ALS and FTD*
**作者**：Nassif, M., Matus, S., Castillo, K., Hetz, C.
**摘要**：通过重组FUS/TLS蛋白模型，探讨自噬通路失调如何促进突变蛋白积累，进而引发神经元死亡，为ALS与额颞叶痴呆(FTD)的共性机制提供依据。
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这些研究均利用重组蛋白技术解析ALS致病蛋白(如SOD1、TDP-43、FUS)的结构功能异常及其病理机制，为治疗策略开发奠定基础。如需更多文献，可检索PubMed或Google Scholar关键词“ALS recombinant protein”。
**Background of ALS-Related Recombinant Proteins**
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder characterized by the loss of motor neurons, leading to muscle weakness, paralysis, and death. Approximately 5-10% of cases are familial (fALS), linked to mutations in genes such as *SOD1*, *TARDBP* (encoding TDP-43), *FUS*, and *C9orf72*. These mutations often result in toxic protein aggregates, misfolding, or dysfunctional RNA metabolism, contributing to neuronal death.
Recombinant proteins corresponding to ALS-associated mutants (e.g., SOD1. TDP-43) are critical tools for studying disease mechanisms. Produced via bacterial, yeast, or mammalian expression systems, these proteins enable researchers to analyze structural changes, aggregation propensity, and interactions with cellular components. For instance, recombinant mutant SOD1 has been used to model oxidative stress and mitochondrial dysfunction in vitro, while TDP-43 variants help elucidate RNA-binding defects and stress granule dynamics.
Additionally, recombinant proteins support therapeutic development. They facilitate high-throughput drug screening to identify aggregation inhibitors or chaperones that stabilize native protein conformations. Antibodies targeting pathological protein forms (e.g., misfolded SOD1) are also designed using recombinant antigens.
Challenges remain, including replicating post-translational modifications and native oligomerization states. Advances in structural biology (e.g., cryo-EM) and gene-editing technologies (e.g., CRISPR) now allow more precise modeling of ALS-related protein pathology. Future research aims to integrate recombinant protein data with patient-derived models (e.g., iPSCs) to bridge molecular insights and clinical translation.
In summary, ALS recombinant proteins are indispensable for decoding pathogenic pathways and accelerating targeted therapies against this devastating disease.
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