| WB | 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 | Beta-mannosidase, 3.2.1.25, Lysosomal beta A mannosidase, Mannanase, Mannase, MANBA, MANB1 |
| Entrez GeneID | 4126 |
| WB Predicted band size | 100.9kDa |
| 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 MANBA antibody is generated from a rabbit immunized with a KLH conjugated synthetic peptide between 712-745 amino acids from human MANBA. |
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以下是关于 **MANBA(β-甘露糖苷酶)抗体** 的3篇参考文献示例(内容基于公开研究概括，非真实文献)：
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1. **文献名称**：*"Development of a Monoclonal Antibody Against Human β-Mannosidase (MANBA) for Lysosomal Storage Disease Diagnosis"*
**作者**：Smith J, et al.
**摘要**：本研究开发了一种特异性识别人类MANBA蛋白的单克隆抗体，并验证了其在溶酶体贮积症(如β-甘露糖苷酶缺乏症)患者组织样本中的诊断应用。该抗体通过免疫印迹和免疫组化证实了其对病理组织中MANBA酶活性缺失的检测能力。
2. **文献名称**：*"MANBA Expression in Colorectal Cancer: A Novel Biomarker Identified by Immunohistochemical Analysis"*
**作者**：Zhang Y, et al.
**摘要**：通过免疫组织化学技术，本文发现MANBA在结直肠癌组织中显著高表达，且与患者预后不良相关。研究使用商业MANBA抗体验证了其作为潜在肿瘤标志物的价值，并探讨了其在糖基化异常调控中的作用。
3. **文献名称**：*"Functional Characterization of β-Mannosidase Knockout Mice Using MANBA-Specific Antibodies"*
**作者**：Johnson R, et al.
**摘要**：利用基因敲除小鼠模型和特异性MANBA抗体，本研究揭示了MANBA缺失导致的中枢神经系统和肝脏中糖脂代谢异常，为β-甘露糖苷酶缺乏症的病理机制提供了直接证据。
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**提示**：如需真实文献，建议通过PubMed或Google Scholar检索关键词“MANBA antibody”、“β-mannosidase immunohistochemistry”等，并筛选近年研究。
MANBA (β-mannosidase) is a lysosomal enzyme encoded by the *MANBA* gene, located on chromosome 4q24. It catalyzes the hydrolysis of β-linked mannose residues from glycoproteins and oligosaccharides, playing a critical role in glycoprotein metabolism. Defects in MANBA due to genetic mutations cause beta-mannosidosis, a rare autosomal recessive lysosomal storage disorder characterized by the accumulation of mannose-rich oligosaccharides. Symptoms range from mild neurological impairments to severe developmental delays, hearing loss, and immune dysfunction, depending on residual enzyme activity.
MANBA antibodies are essential tools for studying the enzyme's expression, localization, and function. They enable the detection of MANBA in tissues and cells via techniques like Western blot, immunohistochemistry, and ELISA, aiding in the diagnosis of beta-mannosidosis. Additionally, these antibodies contribute to research on disease mechanisms, including lysosomal dysfunction and substrate accumulation. Some studies explore their therapeutic potential, such as enzyme replacement therapies or antibody-based targeting for drug delivery.
The development of high-specificity MANBA antibodies has advanced understanding of glycoprotein degradation pathways and lysosomal biology. Ongoing research focuses on correlating MANBA variants with clinical phenotypes and optimizing antibody-based assays for early diagnosis and monitoring of beta-mannosidosis.
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