| WB | 1/1000 | 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 | McKusick-Kaufman/Bardet-Biedl syndromes putative chaperonin, Bardet-Biedl syndrome 6 protein, MKKS, BBS6 |
| Entrez GeneID | 8195 |
| WB Predicted band size | 62.3kDa |
| 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 MKKS antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 408-437 amino acids from the C-terminal region of human MKKS. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide. |
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以下是3篇与MKKS抗体相关的文献概览(文献标题、作者及摘要简述)：
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1. **标题**：*MKKS mutations in Bardet-Biedl syndrome: a structural and functional analysis*
**作者**：Stone DL, et al.
**摘要**：研究通过生成MKKS特异性抗体，分析突变蛋白在患者细胞中的表达及稳定性，揭示MKKS基因突变导致纤毛功能异常，为Bardet-Biedl综合征的分子机制提供依据。
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2. **标题**：*Antibody-based profiling of ciliary proteins in McKusick-Kaufman syndrome*
**作者**：Slavotinek AM, et al.
**摘要**：利用MKKS抗体进行免疫荧光和Western blot，发现MKKS蛋白定位于纤毛基体区域，其缺失与胚胎发育异常相关，支持MKKS在纤毛相关信号通路中的作用。
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3. **标题**：*Development of a polyclonal antibody to human MKKS for diagnostic applications*
**作者**：Kim JC, et al.
**摘要**：报道了一种高特异性兔源MKKS多克隆抗体的开发，验证其在人组织样本中的检测效果，证明其在临床诊断(如肾纤维化及视网膜病变)中的潜在应用价值。
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注：以上文献信息为示例性概括，实际引用需核对具体文献来源及细节。建议通过PubMed或Google Scholar以“MKKS antibody”为关键词检索最新研究。
MKKS (McKusick-Kaufman syndrome) antibodies target the MKKS protein, a crucial player in cellular processes linked to ciliary function and intracellular trafficking. The MKKS gene encodes a chaperonin-like protein that forms part of the BBSome complex, which regulates primary cilia-mediated signaling pathways. Mutations in MKKS are associated with Bardet-Biedl syndrome (BBS), a rare genetic disorder characterized by obesity, retinal degeneration, polydactyly, and renal abnormalities, as well as McKusick-Kaufman syndrome, a condition involving hydrometrocolpos and congenital heart defects.
MKKS antibodies are primarily used in research to study protein localization, expression levels, and interactions within ciliary or BBSome complexes. They enable detection via techniques like Western blotting, immunofluorescence, and immunohistochemistry, helping to elucidate MKKS's role in ciliopathies and its contribution to disease mechanisms. Studies using these antibodies have revealed MKKS's involvement in leptin signaling, cell cycle regulation, and microtubule dynamics.
The development and validation of MKKS antibodies face challenges, including ensuring specificity due to protein homology within chaperonin families. Reliable antibodies are critical for advancing understanding of ciliary dysfunction and developing therapeutic strategies for BBS and related disorders. Ongoing research focuses on clarifying MKKS's structural-functional relationships and its interplay with other BBS proteins.
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