| WB | 咨询技术 | 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 | 7-dehydrocholesterol reductase, 7-DHC reductase, Putative sterol reductase SR-2, Sterol Delta(7)-reductase, DHCR7, D7SR |
| Entrez GeneID | 1717 |
| WB Predicted band size | 54.5kDa |
| 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 | This DHCR7 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 437-463 amino acids from the C-terminal region of human DHCR7. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide. |
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以下是关于DHCR7抗体的示例参考文献(内容为示例性概括，实际文献需通过学术数据库查询)：
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1. **标题**：*"Characterization of DHCR7 Antibodies in the Diagnosis of Smith-Lemli-Opitz Syndrome"*
**作者**：Waterham, H.R., et al.
**摘要**：研究开发了特异性识别DHCR7蛋白的多克隆抗体，用于检测Smith-Lemli-Opitz综合征(SLOS)患者的酶表达缺失。通过Western blot和免疫组化证实抗体在突变细胞系中的敏感性，为SLOS的临床诊断提供工具。
2. **标题**：*"Monoclonal Antibodies Against DHCR7 Reveal Its Role in Cholesterol Biosynthesis"*
**作者**：Correa-Cerro, L.S., Porter, F.D.
**摘要**：利用单克隆抗体研究DHCR7在胆固醇合成途径中的酶活性及亚细胞定位，发现其突变导致7-脱氢胆固醇积累，并探讨抗体在功能研究和药物筛选中的应用潜力。
3. **标题**：*"DHCR7 Enzyme Localization and Regulation: Insights from Immunofluorescence Studies"*
**作者**：Sharpe, L.J., Brown, A.J.
**摘要**：通过DHCR7特异性抗体进行免疫荧光染色，揭示该酶在内质网的分布及其受胆固醇水平调控的机制，为代谢疾病研究提供新方向。
4. **标题**：*"Antibody-Based Detection of DHCR7 Mutations in Metabolic Disorders"*
**作者**：Yu, H., Patel, S.B.
**摘要**：评估多种DHCR7抗体对常见突变体的识别能力，发现部分抗体可区分野生型与突变型蛋白，支持其在遗传性代谢病分子分型中的价值。
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**注**：以上为示例文献，实际研究需参考PubMed、Google Scholar等平台的真实论文(可搜索关键词：DHCR7 antibody, Smith-Lemli-Opitz syndrome, cholesterol biosynthesis)。
**Background of DHCR7 Antibody**
The **7-dehydrocholesterol reductase (DHCR7)** enzyme catalyzes the final step in cholesterol biosynthesis, reducing 7-dehydrocholesterol (7-DHC) to cholesterol. This reaction is critical for maintaining cellular cholesterol homeostasis and synthesizing steroid hormones, vitamin D, and bile acids. Mutations in the *DHCR7* gene disrupt cholesterol metabolism, leading to **Smith-Lemli-Opitz syndrome (SLOS)**, a rare autosomal recessive disorder characterized by developmental abnormalities, intellectual disability, and dysmorphic features.
**DHCR7 antibodies** are immunological tools designed to detect and quantify DHCR7 protein expression in research applications. These antibodies are essential for studying DHCR7’s role in cholesterol biosynthesis, cellular signaling, and disease mechanisms. They are widely used in techniques like Western blotting, immunohistochemistry (IHC), and immunofluorescence (IF) to analyze protein levels, tissue distribution, and subcellular localization.
Research utilizing DHCR7 antibodies has expanded insights into SLOS pathophysiology, including the toxic accumulation of 7-DHC and its oxidative derivatives, which contribute to oxidative stress and developmental defects. Additionally, DHCR7 antibodies aid in exploring links between cholesterol metabolism and broader conditions, such as cancer, neurodegenerative diseases, and lipid disorders. Their specificity and reliability make them pivotal for both basic research and potential therapeutic development targeting DHCR7-related pathways.
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