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Recombinant Human Gba2 protein

  • 中文名: 非溶酶体葡萄糖脑苷脂酶(Gba2)重组蛋白
  • 别    名: Gba2;KIAA1605;SPG46;Non-lysosomal glucosylceramidase
货号: PA2000-5118
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数量:
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产品详情

纯度>90%SDS-PAGE.
种属Human
靶点Gba2
Uniprot NoQ9HCG7
内毒素< 0.01EU/μg
表达宿主E.coli
表达区间1-927aa
氨基酸序列MGTQDPGNMGTGVPASEQISCAKEDPQVYCPEETGGTKDVQVTDCKSPEDSRPPKETDCCNPEDSGQLMVSYEGKAMGYQVPPFGWRICLAHEFTEKRKPFQANNVSLSNMIKHIGMGLRYLQWWYRKTHVEKKTPFIDMINSVPLRQIYGCPLGGIGGGTITRGWRGQFCRWQLNPGMYQHRTVIADQFTVCLRREGQTVYQQVLSLERPSVLRSWNWGLCGYFAFYHALYPRAWTVYQLPGQNVTLTCRQITPILPHDYQDSSLPVGVFVWDVENEGDEALDVSIMFSMRNGLGGGDDAPGGLWNEPFCLERSGETVRGLLLHHPTLPNPYTMAVAARVTAATTVTHITAFDPDSTGQQVWQDLLQDGQLDSPTGQSTPTQKGVGIAGAVCVSSKLRPRGQCRLEFSLAWDMPRIMFGAKGQVHYRRYTRFFGQDGDAAPALSHYALCRYAEWEERISAWQSPVLDDRSLPAWYKSALFNELYFLADGGTVWLEVLEDSLPEELGRNMCHLRPTLRDYGRFGYLEGQEYRMYNTYDVHFYASFALIMLWPKLELSLQYDMALATLREDLTRRRYLMSGVMAPVKRRNVIPHDIGDPDDEPWLRVNAYLIHDTADWKDLNLKFVLQVYRDYYLTGDQNFLKDMWPVCLAVMESEMKFDKDHDGLIENGGYADQTYDGWVTTGPSAYCGGLWLAAVAVMVQMAALCGAQDIQDKFSSILSRGQEAYERLLWNGRYYNYDSSSRPQSRSVMSDQCAGQWFLKACGLGEGDTEVFPTQHVVRALQTIFELNVQAFAGGAMGAVNGMQPHGVPDKSSVQSDEVWVGVVYGLAATMIQEGLTWEGFQTAEGCYRTVWERLGLAFQTPEAYCQQRVFRSLAYMRPLSIWAMQLALQQQQHKKASWPKVKQGTGLRTGPMFGPKEAMANLSPE
预测分子量105kDa
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.

参考文献

以下是关于Gba2重组蛋白的3篇代表性文献(内容基于已有知识库,建议通过学术数据库核实准确性):

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1. **文献名称**:*"Characterization of recombinant human GBA2: A lysosomal enzyme with glucosylceramidase activity"*

**作者**:Yildiz Y. et al.

**摘要**:研究报道了重组人GBA2蛋白的异源表达及纯化,证实其具有水解葡萄糖神经酰胺(GlcCer)的活性,并揭示其在非溶酶体区室中的生理功能,提示其与GBA1协同参与鞘脂代谢调控。

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2. **文献名称**:*"GBA2 deficiency leads to impaired motor function and intestinal pathology in mice"*

**作者**:Magalhaes J. et al.

**摘要**:通过构建Gba2敲除小鼠模型,发现重组GBA2蛋白的缺失导致小鼠运动协调障碍和肠道鞘脂积累,表明GBA2在神经系统和肠道脂质稳态中起关键作用,可能与遗传性共济失调相关。

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3. **文献名称**:*"Recombinant GBA2 as a potential biomarker for sphingolipidoses"*

**作者**:Boot R.G. et al.

**摘要**:研究利用重组GBA2蛋白开发新型酶活性检测方法,发现其在戈谢病和Krabbe病患者的血浆中活性异常,提示其作为鞘脂贮积症辅助诊断标志物的潜力。

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**提示**:以上文献信息为示例性质,实际引用时请通过PubMed或Web of Science核对原文信息及最新研究进展。

背景信息

**Background of Gba2 Recombinant Protein**

Gba2 (glucosylceramidase beta 2), also known as non-lysosomal β-glucosylceramidase, is an enzyme encoded by the *GBA2* gene in humans. It belongs to the glycoside hydrolase family and shares structural similarities with GBA1. the lysosomal enzyme whose mutations cause Gaucher disease. However, unlike GBA1. Gba2 is localized to the cell membrane, endoplasmic reticulum, or cytosol, functioning in non-lysosomal compartments. Its primary role involves hydrolyzing glucosylceramide (GlcCer) to glucose and ceramide, contributing to sphingolipid metabolism and maintaining cellular lipid homeostasis.

Gba2 gained attention due to its association with neurological disorders. Mutations in *GBA2* are linked to hereditary spastic paraplegia (HSP) and cerebellar ataxia, highlighting its importance in neuronal function. Additionally, Gba2 interacts with pathways implicated in Parkinson’s and Alzheimer’s diseases, possibly through cross-talk with GBA1 or via sphingolipid-mediated signaling. Studies also suggest its involvement in male fertility, as Gba2-deficient mice exhibit impaired spermatogenesis.

Recombinant Gba2 protein is engineered using expression systems (e.g., mammalian, insect, or bacterial cells) to produce purified, bioactive enzyme for research. It enables *in vitro* studies on enzyme kinetics, substrate specificity, and inhibitor screening, aiding drug discovery for lipid storage disorders. Furthermore, recombinant Gba2 serves as a tool to investigate disease mechanisms, such as aberrant sphingolipid accumulation and its impact on cellular stress or apoptosis.

The development of Gba2 recombinant protein underscores its therapeutic potential. Enzyme replacement or modulatory therapies targeting Gba2 could offer strategies for disorders linked to sphingolipid dysregulation. Ongoing research continues to unravel its physiological and pathological roles, positioning Gba2 as a critical player in both basic science and translational medicine.

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