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 | Rab proteins geranylgeranyltransferase component A 1, Choroideremia protein, Rab escort protein 1, REP-1, TCD protein, CHM, REP1, TCD |
Entrez GeneID | 1121 |
WB Predicted band size | 73.5kDa |
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 |
Immunogen | This CHM antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 292-320 amino acids from the Central region of human CHM. |
Formulation | Purified antibody in PBS with 0.05% sodium azide. |
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以下是3篇关于CHM抗体的示例文献(注:文献为假设性示例,实际研究请以真实数据库检索结果为准):
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1. **文献名称**: *Autoantibodies against CHM Protein in Retinal Degenerative Diseases*
**作者**: Smith A et al.
**摘要**: 研究报道在视网膜退行性疾病患者中发现针对CHM基因编码蛋白(Rab escort protein-1. REP-1)的自身抗体,提示其可能与Choroideremia(CHM)病理机制相关,为疾病诊断提供了潜在生物标志物。
2. **文献名称**: *Development of a High-Specificity Monoclonal Antibody for CHM Gene Product Detection*
**作者**: Zhang L et al.
**摘要**: 描述了一种新型单克隆抗体的开发,该抗体特异性识别CHM基因表达的REP-1蛋白,可用于体外组织样本的免疫组化检测,助力CHM相关疾病的分子诊断研究。
3. **文献名称**: *CHM Antibody-Mediated Immune Response in Gene Therapy for Ocular Disorders*
**作者**: Johnson R et al.
**摘要**: 探讨腺相关病毒(AAV)载体介导的CHM基因治疗中,患者体内可能产生的抗CHM抗体对治疗效果的潜在影响,强调免疫监测在基因治疗中的重要性。
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如需实际文献,建议通过PubMed或Google Scholar以关键词“CHM antibody”、“Choroideremia REP-1 antibody”进行检索。
Choroideremia (CHM) is a rare X-linked recessive retinal degenerative disease caused by mutations in the *CHM* gene, which encodes Rab escort protein 1 (REP1). This protein plays a critical role in lipid modification (prenylation) of Rab GTPases, essential for intracellular vesicle trafficking and membrane dynamics. In CHM, REP1 dysfunction leads to impaired Rab protein activity, resulting in progressive degeneration of retinal pigment epithelium, photoreceptors, and choroid, ultimately causing vision loss.
CHM antibodies, specifically targeting the REP1 protein or related biomarkers, are vital tools in research and diagnostics. They enable detection of REP1 expression levels in patient samples, aiding in confirming CHM diagnoses and studying disease mechanisms. Additionally, these antibodies are used in preclinical studies to evaluate gene therapy approaches, such as adeno-associated virus (AAV)-mediated *CHM* gene replacement, which aims to restore REP1 function. Recent advances in CHM research highlight the potential of antibody-based assays to monitor therapeutic efficacy and biomarker changes in clinical trials.
Despite progress, challenges remain in understanding tissue-specific REP1 interactions and optimizing treatments. CHM antibodies continue to facilitate exploration of molecular pathways and therapeutic strategies, offering hope for halting or reversing retinal degeneration in affected individuals.
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