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Rabbit Polyclonal TOR1AIP1 Antibody

  • 中文名: TOR1AIP1抗体
  • 别    名: LAP1; LAP1B; LGMD2Y
货号: IPDX10174
Price: ¥1180
数量:
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验证与应用

应用及物种
WB 咨询技术 Human,Mouse,Rat
IF 咨询技术 Human,Mouse,Rat
IHC 1/50-1/300 Human,Mouse,Rat
ICC 技术咨询 Human,Mouse,Rat
FCM 咨询技术 Human,Mouse,Rat
Elisa 1/5000-1/10000 Human,Mouse,Rat

产品详情

AliasesLAP1; LAP1B; LGMD2Y
Host/IsotypeRabbit IgG
Antibody TypePrimary antibody
StorageStore at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species ReactivityHuman, Mouse, Rat
ImmunogenFusion protein of human TOR1AIP1
FormulationPurified antibody in PBS with 0.05% sodium azide and 50% glycerol.

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参考文献

以下是关于TOR1AIP1抗体的3篇参考文献及其简要摘要:

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1. **文献名称**: *"TOR1AIP1 mutations cause nuclear envelope defects and disrupt cellular homeostasis"*

**作者**: Jung JH, et al.

**摘要**: 该研究通过免疫荧光和Western blot技术,利用TOR1AIP1抗体揭示了其在维持核膜完整性中的作用。实验表明,TOR1AIP1缺失导致核形态异常和内质网应激反应激活,提示其在细胞稳态中的关键功能。

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2. **文献名称**: *"Mutations in TOR1AIP1 encoding LAP1B cause a novel disorder of nuclear envelope with severe muscular dystrophy"*

**作者**: Denecke J, et al.

**摘要**: 本研究在肌肉营养不良症患者中鉴定出TOR1AIP1基因突变,并通过抗体检测发现突变导致LAP1B蛋白表达显著降低,证实其与核膜功能障碍及肌肉退化的直接关联。

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3. **文献名称**: *"The interaction between TorsinA and TOR1AIP1: implications for nuclear envelope dynamics"*

**作者**: Goodchild RE, et al.

**摘要**: 研究利用TOR1AIP1抗体进行免疫共沉淀实验,揭示其与TorsinA蛋白的相互作用机制,证明两者共同调控核膜-内质网间的囊泡运输,为神经退行性疾病提供分子机制解释。

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注:上述文献信息为示例性质,实际文献可能需要通过学术数据库(如PubMed、Web of Science)进一步验证。如需具体文献,建议结合关键词“TOR1AIP1 antibody”或“LAP1B antibody”检索最新研究。

背景信息

The TOR1AIP1 antibody targets the Torsin-1A-interacting protein 1 (TOR1AIP1), also known as lamina-associated polypeptide 1 (LAP1), a nuclear envelope protein encoded by the TOR1AIP1 gene. LAP1 interacts with TorsinA, a AAA+ ATPase linked to early-onset dystonia (DYT1) when mutated. This interaction is critical for maintaining nuclear envelope structure, regulating endoplasmic reticulum-nuclear envelope communication, and influencing cellular processes like chromatin organization and mechanotransduction. TOR1AIP1 mutations are associated with severe musculoskeletal disorders, including congenital muscular dystrophy with nuclear envelopathies, highlighting its role in muscle and neuronal homeostasis. Antibodies against TOR1AIP1 are essential tools for studying its expression, localization, and function in disease models. They enable detection via techniques like Western blotting, immunofluorescence, and immunohistochemistry, aiding research into nuclear envelope dynamics, TorsinA-related pathologies, and mechanisms underlying muscle degeneration. Recent studies also explore LAP1's involvement in cellular stress responses and viral infection pathways. Validated TOR1AIP1 antibodies help dissect molecular pathways in genetic disorders and potential therapeutic targets, bridging gaps between basic research and clinical understanding of nuclear envelopathies.

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