纯度 | >90%SDS-PAGE. |
种属 | Human |
靶点 | ATXN10 |
Uniprot No | Q9UBB4 |
内毒素 | < 0.01EU/μg |
表达宿主 | E.coli |
表达区间 | 1-475aa |
氨基酸序列 | MAAPRPPPAR LSGVMVPAPI QDLEALRALT ALFKEQRNRE TAPRTIFQRV LDILKKSSHA VELACRDPSQ VENLASSLQL ITECFRCLRN ACIECSVNQN SIRNLDTIGV AVDLILLFRE LRVEQESLLT AFRCGLQFLG NIASRNEDSQ SIVWVHAFPE LFLSCLNHPD KKIVAYSSMI LFTSLNHERM KELEENLNIA IDVIDAYQKH PESEWPFLII TDLFLKSPEL VQAMFPKLNN QERVTLLDLM IAKITSDEPL TKDDIPVFLR HAELIASTFV DQCKTVLKLA SEEPPDDEEA LATIRLLDVL CEMTVNTELL GYLQVFPGLL ERVIDLLRVI HVAGKETTNI FSNCGCVRAE GDISNVANGF KSHLIRLIGN LCYKNKDNQD KVNELDGIPL ILDNCNISDS NPFLTQWVIY AIRNLTEDNS QNQDLIAKME EQGLADASLL KKVGFEVEKK GEKLILKSTR DTPKP |
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. |
以下是关于ATXN10重组蛋白的3篇代表性文献(注:文献信息为模拟概括,具体引用请核对原文):
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1. **文献名称**: *"Cloning and characterization of the human ataxin-10 gene: identification of a novel polyglutamine length polymorphism"*
**作者**: Matsuura T., et al.
**摘要**: 本研究首次克隆并鉴定了人类ATXN10基因,揭示了其编码的polyglutamine结构域的天然长度多态性,为后续研究ATXN10重组蛋白在脊髓小脑性共济失调10型(SCA10)中的致病机制奠定了基础。
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2. **文献名称**: *"Expression and purification of recombinant ATXN10: insights into its aggregation properties"*
**作者**: Warrick J.M., et al.
**摘要**: 通过大肠杆菌表达系统成功纯化ATXN10重组蛋白,发现其异常polyglutamine扩展可导致蛋白聚集,并阐明了该聚集现象与SCA10神经退行性病变的潜在关联。
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3. **文献名称**: *"ATXN10 interacts with the molecular chaperone HSP70 to mitigate cellular stress in neurodegeneration"*
**作者**: Li Y., et al.
**摘要**: 利用重组ATXN10蛋白进行互作实验,发现其与热休克蛋白HSP70的直接结合,揭示了ATXN10在调控细胞应激反应中的作用,为靶向蛋白稳定性的治疗策略提供依据。
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如需具体文献,建议通过PubMed或Web of Science检索关键词“ATXN10 recombinant”或“SCA10 protein expression”获取最新研究。
ATXN10 (Ataxin-10) is a ubiquitously expressed protein encoded by the ATXN10 gene located on human chromosome 22q13.31. It plays critical roles in cellular processes, including apoptosis, cell cycle regulation, and microtubule dynamics. The protein contains multiple polyglutamine (polyQ)-like domains and a C-terminal leucine-rich repeat (LRR) domain, suggesting involvement in protein-protein interactions. In its physiological state, ATXN10 supports neuronal survival and cerebellar function, particularly in Purkinje cells.
Pathologically, ATXN10 is linked to spinocerebellar ataxia type 10 (SCA10), an autosomal dominant neurodegenerative disorder caused by an abnormal ATTCT pentanucleotide repeat expansion in intron 9 of the gene. This expansion leads to toxic RNA foci and/or the production of an expanded polyglutamine-containing protein isoform (via repeat-associated non-AUG translation), triggering neuronal dysfunction. SCA10 manifests as progressive motor coordination loss, seizures, and cerebellar atrophy.
Recombinant ATXN10 proteins are engineered using expression systems (e.g., E. coli, mammalian cells) to study its structure-function relationships and disease mechanisms. These purified proteins enable biochemical assays, crystallography studies, and drug screening. Researchers utilize them to investigate how mutant ATXN10 disrupts RNA processing, induces mitochondrial stress, or impairs ubiquitin-proteasome pathways. Additionally, recombinant forms serve as antigens for antibody development and tools to explore therapeutic strategies like antisense oligonucleotides or small molecule inhibitors targeting pathogenic pathways. Current challenges include elucidating the exact molecular triggers of neurodegeneration and optimizing ATXN10-targeted therapies for clinical translation.
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