| WB | 1/1000-1/2000 | 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 | UPS; PBGD; PORC; PBG-D; HMBS; Hydroxymethylbilane synthase;;Porphobilinogen deaminase |
| WB Predicted band size | Calculated MW: 39 kDa ; Observed MW: 42 kDa |
| 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,Mouse,Rat |
| Immunogen | A synthesized peptide derived from human Porphobilinogen deaminase |
| Formulation | Purified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol. |
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以下是关于HMBS抗体的3篇参考文献摘要概括：
1. **文献名称**：*Production and characterization of monoclonal antibodies against human hydroxymethylbilane synthase*
**作者**：Astrin KH, Warner CA, Yoo HW, et al.
**摘要**：该研究报道了针对人HMBS蛋白的单克隆抗体的开发与表征，验证了抗体在免疫印迹和免疫组化中的应用，用于检测不同组织及细胞系中HMBS的表达水平，为卟啉症相关研究提供工具。
2. **文献名称**：*Molecular heterogeneity of acute intermittent porphyria: mutations in the HMBS gene and functional analysis of expressed mutant enzymes*
**作者**：Chen B, Solis-Villa C, Hakenberg J, et al.
**摘要**：通过构建HMBS基因突变体并利用特异性抗体检测突变蛋白的表达与活性，揭示了急性间歇性卟啉症(AIP)患者中HMBS酶功能缺陷的分子机制，强调了抗体在突变蛋白功能研究中的重要性。
3. **文献名称**：*Immunohistochemical localization of hydroxymethylbilane synthase in normal and porphyric human liver*
**作者**：Solis C, Anderson KE, Sassa S, et al.
**摘要**：使用HMBS特异性抗体对正常及卟啉症患者的肝组织进行免疫组化分析，发现HMBS蛋白在肝细胞中的定位异常与疾病进展相关，为病理机制研究提供依据。
以上文献聚焦于HMBS抗体的开发、应用及其在疾病机制与诊断中的价值。
The hydroxymethylbilane synthase (HMBS) antibody is a tool used to study the HMBS enzyme, a critical component in heme biosynthesis. HMBS, also known as porphobilinogen deaminase (PBGD), catalyzes the polymerization of four porphobilinogen molecules into hydroxymethylbilane, a linear tetrapyrrole precursor for heme and chlorophyll. Defects in the HMBS gene lead to acute intermittent porphyria (AIP), an autosomal dominant metabolic disorder characterized by the accumulation of neurotoxic porphyrin precursors.
HMBS antibodies are widely employed in research to detect and quantify HMBS protein expression in tissues or cell lines, aiding in the study of porphyria pathogenesis and enzyme dysfunction. They are utilized in techniques like Western blotting, immunohistochemistry, and immunofluorescence to assess HMBS localization and expression levels. In clinical diagnostics, these antibodies may help confirm AIP diagnosis by identifying reduced HMBS activity or mutations.
Recent studies also explore HMBS's role beyond heme synthesis, including its potential involvement in oxidative stress responses and cancer progression. The development of HMBS antibodies has advanced understanding of enzyme regulation, substrate binding, and interactions with other heme biosynthesis pathway components. Their specificity and reliability make them indispensable for both basic research and translational applications targeting porphyrias and related metabolic disorders.
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