| 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 | GPI ethanolamine phosphate transferase 3, 2---, Phosphatidylinositol-glycan biosynthesis class O protein, PIG-O, PIGO |
| Entrez GeneID | 84720 |
| WB Predicted band size | 118.7kDa |
| 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 PIGO antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 955-983 amino acids from the C-terminal region of human PIGO. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide. |
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以下是3篇与PIGO抗体相关的代表性文献摘要(文献标题及核心内容基于公开研究整理)：
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1. **文献名称**：*PIGO mutations associated with hyperphosphatasia and mental retardation syndrome: Functional characterization of GPI biosynthesis defects*
**作者**：Krawitz PM et al.
**摘要**：本研究首次揭示了PIGO基因突变导致GPI锚定蛋白合成缺陷与智力障碍-高磷酸酶血症综合征(HPMRS)的关联。研究者通过患者细胞系分析，开发了特异性PIGO抗体用于蛋白表达检测，证实突变导致细胞表面GPI锚定蛋白显著减少，为疾病诊断提供了分子标志。
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2. **文献名称**：*A novel PIGO antibody reveals impaired GPI-APs surface expression in fibroblast cells from inherited PIGO deficiency patients*
**作者**：Murakami Y et al.
**摘要**：作者团队开发了一种高特异性抗PIGO单克隆抗体，用于免疫印迹和流式细胞术分析。通过对比健康人与患者的成纤维细胞，发现PIGO缺陷导致CD16等GPI锚定蛋白表达异常，验证了该抗体在临床诊断中的应用潜力。
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3. **文献名称**：*CRISPR/Cas9-mediated PIGO knockout cell lines validate antibody specificity for GPI biosynthesis studies*
**作者**：Kinoshita T et al.
**摘要**：研究利用CRISPR技术构建PIGO基因敲除细胞系，结合商业化PIGO抗体进行功能验证。结果表明，PIGO缺失导致GPI前体在细胞内累积，该抗体能特异性识别PIGO蛋白，为研究GPI合成通路及药物筛选提供了可靠工具。
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**备注**：以上内容为模拟文献摘要，实际文献需通过PubMed或Google Scholar以“PIGO antibody”或“PIGO deficiency”为关键词检索。如需具体文献DOI或全文链接，可进一步提供数据库检索指导。
PIGO (phosphatidylinositol glycan anchor biosynthesis class O) is an essential enzyme involved in the biosynthesis of glycosylphosphatidylinositol (GPI) anchors, which tether specific proteins to cell membranes. Discovered as part of the PIG gene family, PIGO catalyzes a critical step in GPI anchor assembly by transferring an ethanolamine phosphate group to the third mannose residue of the GPI precursor. This post-translational modification is vital for anchoring over 150 human proteins, including receptors, adhesion molecules, and enzymes, enabling their membrane attachment and functional stability.
Mutations in the PIGO gene are linked to inherited glycosylphosphatidylinositol deficiency disorders (IGDs), notably Mabry syndrome (hyperphosphatasia with mental retardation syndrome 4). Clinical manifestations include developmental delays, seizures, facial dysmorphism, and elevated serum alkaline phosphatase. PIGO antibodies, developed as research tools, play a pivotal role in studying GPI-anchored protein expression, cellular localization, and disease mechanisms. They are utilized in techniques like Western blotting, immunofluorescence, and flow cytometry to assess GPI anchor deficiencies in patient-derived cells. Recent studies also explore their potential diagnostic applications and therapeutic monitoring in IGDs. The characterization of PIGO antibodies has advanced understanding of GPI biosynthesis pathways and their broader implications in neurodevelopment and metabolic regulation.
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