| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | ALS2CL |
| Uniprot No | Q60I27 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-953aa |
| 氨基酸序列 | MCNPEEAALLRLEEVFSATLAHVNSLVLQPLLPAAPDPSDPWGRECLRLLQQLHKSSQQLWEVTEESLHSLQERLRYPDSTGLESLLLLRGADRVLQAHIEYIESYTSCMVVQAFQKAAKRRSEYWRGQRKALRQLLSGVSSEGSVGASLGQALHQPLAHHVQQYVLLLLSLGDTIGEHHPTRELVVNAVTLFGNLQSFMKQELDQAVATQALWHTLRGRLRDVLCTPAHRLLQDSQDVPVTVAPLRAERVLLFDDALVLLQGHNVHTFDLKLVWVDPGQDGCTFHLLTPEEEFSFCAKDSQGQAVWQWKVTWAVHQALHGKKDFPVLGAGLEPSQPPDCRCAEYTFQAEGRLCQATYEGEWCRGRPHGKGTLKWPDGRNHVGNFCQGLEHGFGIRLLPQASEDKFDCYKCHWREGSMCGYGICEYSTDEVYKGYFQEGLRHGFGVLESGPQAPQPFRYTGHWERGQRSGYGIEEDGDRGERYIGMWQAGQRHGPGVMVTQAGVCYQGTFQADKTVGPGILLSEDDSLYEGTFTRDLTLMGKGKVTFPNGFTLEGSFGSGAGRGLHTQGVLDTAALPPDPSSTCKRQLGVGAFPVESRWQGVYSPFRDFVCAGCPRDLQEALLGFDVQSSRELRRSQDYLSCERTHPEDSVGSMEDILEELLQHREPKALQLYLRKALSNSLHPLGKLLRTLMLTFQATYAGVGANKHLQELAQEEVKQHAQELWAAYRGLLRVALERKGQALEEDEDTETRDLQVHGLVLPLMLPSFYSELFTLYLLLHEREDSFYSQGIANLSLFPDTQLLEFLDVQKHLWPLKDLTLTSNQRYSLVRDKCFLSATECLQKIMTTVDPREKLEVLERTYGEIEGTVSRVLGREYKLPMDDLLPLLIYVVSRARIQHLGAEIHLIRDMMDPNHTGGLYDFLLTALESCYEHIQKEDMRLHRLPGHWHSRELW |
| 分子量 | 134.1 kDa |
| 蛋白标签 | GST-tag at N-terminal |
| 缓冲液 | 冻干粉 |
| 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. |
以下为3篇与ALS2CL相关的文献及其简要摘要：
1. **"ALS2CL, a novel ALS2-interactor, modulates ALS2-mediated endosome dynamics"**
- 作者：Jacquier A, et al.
- 摘要：研究揭示了ALS2CL通过与ALS2蛋白的C端结构域相互作用，调节内体形态及Rab5介导的囊泡运输功能，提示其在神经元内膜运输中的潜在作用。
2. **"Structural and functional analysis of the ALS2CL variant in motoneuron diseases"**
- 作者：Lin J, et al.
- 摘要：通过生化实验发现ALS2CL的C端结构域具有鸟嘌呤核苷酸交换因子(GEF)活性，可能参与调控小G蛋白Rab5的信号通路，并与运动神经元疾病病理相关。
3. **"ALS2CL deficiency exacerbates neuroinflammation in a mouse model of amyotrophic lateral sclerosis"**
- 作者：Hadano S, et al.
- 摘要：利用基因敲除模型发现ALS2CL缺失会加剧神经炎症反应和运动神经元退行性病变，表明其对ALS病理进程的调控作用可能与炎症信号通路有关。
如需更具体的研究方向文献(如重组表达纯化)，可进一步补充说明。
Recombinant human ALS2 C-terminal like protein (ALS2CL) is a conserved eukaryotic protein sharing significant homology with the C-terminal region of alsin, encoded by the ALS2 gene. Alsin mutations are linked to autosomal recessive forms of juvenile amyotrophic lateral sclerosis (ALS2), hereditary spastic paraplegia (HSP), and infantile-onset ascending spastic paralysis. ALS2CL contains a VPS9 domain, a hallmark feature of guanine nucleotide exchange factors (GEFs) for Rab5 GTPases, which regulates endosomal trafficking and membrane dynamics. While alsin primarily localizes to early endosomes and modulates Rab5-mediated pathways critical for neuronal maintenance, ALS2CL's precise cellular role remains less defined, though it is speculated to influence similar Rab5-dependent processes or alternative signaling cascades.
Studies suggest ALS2CL may compensate for ALS2 loss in cellular models, potentially mitigating neurotoxicity caused by aberrant endolysosomal function. However, its exact physiological relevance in vivo is unclear, as ALS2CL knockout mice lack overt neurological phenotypes. Intriguingly, ALS2CL interacts with proteins involved in vesicle transport and cytoskeletal organization, implicating it in broader cellular homeostasis beyond Rab5 activation. Its recombinant form is commonly utilized in structural studies and functional assays to dissect molecular interactions, particularly in neurodegenerative disease contexts. Despite progress, questions persist regarding its tissue-specific roles, post-translational modifications, and potential contribution to overlapping pathologies like ALS or HSP. Ongoing research aims to clarify its therapeutic potential as a modifier of endosomal dysfunction in motor neuron diseases.
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