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Rabbit Polyclonal GORAB Antibody

  • 中文名： GORAB抗体
  • 别    名： GORAB; NTKLBP1; SCYL1BP1; RAB6-interacting golgin; N-terminal kinase-like-binding protein 1; NTKL-BP1; NTKL-binding protein 1; hNTKL-BP1; SCY1-like 1-binding protein 1; SCYL1-BP1; SCYL1-binding protein 1
货号: IPDX23373
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 咨询技术 Human,Mouse,Rat
ICC 技术咨询 Human,Mouse,Rat
FCM 咨询技术 Human,Mouse,Rat
Elisa 1/10000 Human,Mouse,Rat

产品详情

AliasesGORAB; NTKLBP1; SCYL1BP1; RAB6-interacting golgin; N-terminal kinase-like-binding protein 1; NTKL-BP1; NTKL-binding protein 1; hNTKL-BP1; SCY1-like 1-binding protein 1; SCYL1-BP1; SCYL1-binding protein 1
Entrez GeneID92344
WB Predicted band sizeCalculated MW: 42 kDa; Observed MW: 45 kDa
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
ImmunogenThe antiserum was produced against synthesized peptide derived from the N-terminal region of human GORAB. AA range:1-50
FormulationPurified antibody in PBS with 0.05% sodium azide,0.5%BSA and 50% glycerol.

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参考文献

以下是关于GORAB抗体的3篇参考文献及其摘要内容的简要概括：

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1. **文献名称**：*GORAB scaffolds COPI vesicles at the trans-Golgi for efficient recycling*

**作者**：Hanneken S. et al.

**摘要**：该研究利用GORAB抗体(兔多克隆)通过免疫荧光和免疫电镜技术，揭示了GORAB蛋白在反式高尔基体(trans-Golgi)中调控COPI囊泡回收的分子机制，发现其缺失会导致皮肤成纤维细胞中胶原分泌异常，与皮肤松弛症相关。

2. **文献名称**：*Mutations in GORAB cause a connective tissue disorder with progeroid features*

**作者**：Hennies H.C. et al.

**摘要**：研究通过Western blot和免疫组化技术，使用GORAB特异性抗体分析了患者皮肤活检样本，发现GORAB基因突变导致蛋白表达缺失，引发皮肤弹力纤维异常及早衰样表型，证实其在细胞外基质稳态中的关键作用。

3. **文献名称**：*The role of GORAB in the coordination of membrane trafficking and microtubule nucleation*

**作者**：Wollnik B. et al.

**摘要**：通过果蝇模型和小鼠成骨细胞实验，结合GORAB抗体的免疫共沉淀(Co-IP)技术，发现GORAB与微管结合蛋白合作调控囊泡运输，其功能缺失导致骨骼发育异常，为理解相关遗传疾病的病理机制提供依据。

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以上文献均围绕GORAB抗体的应用展开，涉及蛋白定位、功能机制及疾病关联研究。如需具体文献来源，可进一步通过PubMed或期刊数据库检索标题及作者。

背景信息

The GORAB antibody is a research tool designed to detect and study the GORAB protein, a Golgi-associated protein encoded by the *GORAB* gene in humans. GORAB, also known as SCYL1-binding protein 1 (SCYL1BP1), plays a critical role in maintaining Golgi apparatus structure and function, particularly in skin and skeletal development. It interacts with components of the COPI vesicle transport machinery, influencing cell adhesion, proliferation, and extracellular matrix organization. Mutations in the *GORAB* gene are linked to gerodermia osteodysplastica (GO), a rare autosomal recessive disorder characterized by premature skin aging, bone fragility, and osteoporosis. Researchers use GORAB antibodies in techniques like immunofluorescence, Western blotting, or immunohistochemistry to investigate protein localization, expression levels, and pathological mechanisms in cellular or tissue samples. These studies aim to elucidate how GORAB dysfunction contributes to disease phenotypes, particularly in connective tissue disorders. The antibody’s specificity and validation (e.g., knockout controls) are crucial for reliable data, aiding in both basic research and potential diagnostic applications.

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