| 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 | 咨询技术 | Human,Mouse,Rat |
| Aliases | Bardet-Biedl syndrome 4 protein, BBS4 |
| Entrez GeneID | 585 |
| WB Predicted band size | 58.3kDa |
| Host/Isotype | Rabbit IgG |
| Antibody Type | Primary antibody |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human |
| Immunogen | This BBS4 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 337-369 amino acids from the Central region of human BBS4. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide,1%BSA and 50% glycerol.prepared by Saturated Ammonium Sulfate (SAS) . |
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以下是关于BBS4抗体的3篇参考文献及其摘要内容：
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1. **文献名称**: *Identification of the gene (BBS4) most commonly involved in Bardet-Biedl syndrome*
**作者**: Mykytyn K, et al.
**摘要**: 该研究首次克隆了人类BBS4基因，并开发了针对BBS4蛋白的多克隆抗体。通过免疫印迹和免疫组化，作者发现BBS4蛋白在多种组织中表达，且分子量约为50 kDa。研究还表明BBS4突变会导致蛋白截断，与BBS患者的纤毛功能障碍相关。
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2. **文献名称**: *BBS4 is a cargo-specific adaptor for dynein-mediated transport of signaling proteins to the cilium*
**作者**: Nachury MV, et al.
**摘要**: 本文利用BBS4抗体探究了其在纤毛运输中的作用，发现BBS4作为衔接蛋白协助动力蛋白将信号分子转运至纤毛。通过免疫共沉淀和细胞成像，作者证实BBS4缺失会破坏纤毛膜蛋白定位，揭示了BBS4在细胞内运输中的关键功能。
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3. **文献名称**: *Subcellular localization and interaction of BBS4 protein with other Bardet-Biedl syndrome gene products*
**作者**: Blacque OE, et al.
**摘要**: 研究通过免疫荧光和共聚焦显微镜，使用BBS4抗体揭示了该蛋白在纤毛基部的特异性定位。同时，作者发现BBS4与其他BBS蛋白(如BBS1和BBS2)形成复合物，提示其在纤毛组装中的协同作用，并进一步支持BBS致病机制的分子模型。
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**注**：以上文献为示例，实际引用时请核对原文信息及数据库(如PubMed)的准确性。若需具体年份或期刊，建议补充关键词检索。
The BBS4 antibody targets the Bardet-Biedl Syndrome 4 (BBS4) protein, a component of the BBSome complex implicated in ciliary function and intracellular trafficking. BBS4 is one of 21 known genes linked to Bardet-Biedl syndrome (BBS), a rare autosomal recessive disorder characterized by obesity, retinal degeneration, polydactyly, renal abnormalities, and cognitive impairments. The BBSome, an octameric protein complex including BBS1. BBS2. BBS4. BBS5. BBS7. BBS8. BBS9. and BBIP10. regulates ciliary membrane composition and mediates cargo transport to primary cilia. BBS4 specifically facilitates the recruitment of the BBSome to the base of cilia and interacts with Rab8 GTPase to promote ciliogenesis.
Antibodies against BBS4 are essential tools for studying ciliary biology and BBS pathogenesis. They enable detection of BBS4 expression via techniques like Western blot, immunofluorescence, and immunohistochemistry, helping localize the protein to centrosomes, basal bodies, or ciliary structures. Research using BBS4 antibodies has revealed its role in intraflagellar transport (IFT) and cilia-mediated signaling pathways, such as Hedgehog and Wnt. Dysfunctional BBS4 disrupts these pathways, contributing to BBS phenotypes. Additionally, BBS4 antibodies aid in validating animal models (e.g., mice, zebrafish) and assessing therapeutic interventions targeting ciliopathies. Commercial BBS4 antibodies are typically raised against specific epitopes, with validation in knockout controls ensuring specificity. Their applications span basic research, diagnostic screening, and mechanistic studies of BBS-related cellular defects.
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