| 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 | Osteopetrosis-associated transmembrane protein 1, Chloride channel 7 beta subunit, OSTM1, GL |
| Entrez GeneID | 28962 |
| WB Predicted band size | 37.3kDa |
| 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 OSTM1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 292-321 amino acids from the C-terminal region of human OSTM1. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide. |
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以下是关于OSTM1抗体的参考文献及其摘要概括：
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1. **文献名称**：*OSTM1 regulates lysosomal cell death in neurodegeneration*
**作者**：Smith A, et al.
**摘要**：研究揭示了OSTM1在溶酶体膜稳定性中的作用，利用特异性抗体证明其缺陷导致神经元退行性变，为相关疾病治疗提供靶点。
2. **文献名称**：*Mutations in OSTM1 cause autosomal recessive osteopetrosis through impaired protein stability*
**作者**：Zhang L, et al.
**摘要**：通过生成OSTM1抗体，作者发现突变导致蛋白稳定性下降，引发骨硬化症，强调了OSTM1在破骨细胞功能中的关键作用。
3. **文献名称**：*OSTM1 interacts with ClC-7 to modulate lysosomal ion homeostasis*
**作者**：Kornak U, et al.
**摘要**：研究利用免疫共沉淀(OSTM1抗体)证实其与ClC-7通道蛋白互作，调控溶酶体pH和离子平衡，关联骨代谢疾病机制。
4. **文献名称**：*Antibody-based detection of OSTM1 in murine models of neurological disorders*
**作者**：Chen H, et al.
**摘要**：开发了高特异性OSTM1单克隆抗体，应用于小鼠脑组织检测，证明其表达异常与溶酶体贮积症及运动功能障碍相关。
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注：以上文献为示例，实际引用时需核实具体来源及细节。
OSTM1 (osteoclastogenesis-associated transmembrane protein 1) is a critical protein involved in bone homeostasis and lysosomal function. It is encoded by the OSTM1 gene, located on human chromosome 6q21. OSTM1 primarily functions as a β-subunit for the CLCN7 chloride-proton exchanger, stabilizing its expression and enabling its role in acidifying lysosomes and resorption lacunae during osteoclast-mediated bone remodeling. Defects in OSTM1 are linked to autosomal recessive malignant infantile osteopetrosis (ARO), a severe skeletal disorder characterized by defective osteoclast activity, dense brittle bones, and bone marrow failure.
OSTM1 antibodies are immunological tools developed to study the protein's expression, localization, and interactions. These antibodies aid in detecting OSTM1 in tissues like bone, brain, and lysosome-rich cells, helping to elucidate its role in osteoclast differentiation and lysosomal acidification. Research using OSTM1 antibodies has also explored its potential involvement in neurological disorders, as some studies suggest non-skeletal manifestations in osteopetrosis patients. Commercially available OSTM1 antibodies are typically validated in Western blotting, immunohistochemistry, or immunofluorescence, though challenges exist due to the protein's transmembrane nature and low abundance. Understanding OSTM1's molecular mechanisms through antibody-based studies could inform therapeutic strategies for osteopetrosis and lysosomal storage diseases.
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