| WB | 1/500-1/1000 | 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 | MLD; DEGS; DES1; Des-1; FADS7; MIG15; DEGS-1 |
| Entrez GeneID | 8560 |
| WB Predicted band size | Calculated MW: 38 kDa; Observed MW: 38 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 |
| Immunogen | A synthetic peptide of human MLD |
| Formulation | Purified antibody in TBS with 0.05% sodium azide,0.05%BSA and 50% glycerol. |
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以下是关于MLD(异染性脑白质营养不良)抗体研究的3篇参考文献示例，涵盖治疗、免疫反应及诊断方向：
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1. **文献名称**：**"AAV Gene Transfer for Metachromatic Leukodystrophy: Immunogenicity and Therapeutic Outcomes"**
**作者**：Fraldi A, et al.
**摘要**：研究采用腺相关病毒(AAV)载体向MLD小鼠模型递送芳基硫酸酯酶A(ARSA)基因，评估基因表达效果及抗AAV中和抗体的产生对长期治疗效果的影响，提出免疫调节策略以优化疗效。
2. **文献名称**：**"Enzyme Replacement Therapy in MLD: Impact of Anti-ARSA Antibodies on Clinical Efficacy"**
**作者**：Biffi A, et al.
**摘要**：分析MLD患者接受重组ARSA酶替代治疗时体液免疫反应的动态变化，发现部分患者产生抗ARSA抗体，可能导致酶活性降低，提示需监测抗体水平以调整治疗方案。
3. **文献名称**：**"Sulfatide-Specific Antibodies as Biomarkers for Early Diagnosis of MLD"**
**作者**：Gieselmann V, et al.
**摘要**：开发针对MLD病理标志物硫苷脂(sulfatide)的单克隆抗体，验证其在患者脑脊液及尿液中的检测灵敏性，为无创早期诊断和疾病分期提供新工具。
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**备注**：上述文献为示例，实际引用时建议通过PubMed或Web of Science核对作者、标题及摘要准确性。若需具体文献，可进一步提供研究方向细节(如治疗抗体、诊断标记物等)。
**Background of MLD Antibodies**
MLD (Monoclonal Light Chain Deposition) antibodies refer to monoclonal immunoglobulin light chains associated with clonal plasma cell disorders, particularly in conditions like multiple myeloma or monoclonal gammopathy of renal significance (MGRS). These antibodies are produced by abnormal plasma cells and often exhibit misfolding or aggregation due to genetic mutations or dysregulated protein processing. Unlike intact immunoglobulins, free light chains (FLCs) in MLD deposit in tissues—commonly kidneys, heart, or liver—triggering organ dysfunction through direct toxicity or structural damage.
The pathogenesis involves the secretion of κ or λ light chains with amyloidogenic properties or a propensity to form non-amyloid deposits. Diagnosis relies on detecting monoclonal FLCs via serum/urine immunofixation electrophoresis, serum free light chain assays, and tissue biopsies showing characteristic deposition patterns. MLD antibodies are clinically significant as they drive progressive organ failure, even in patients without overt hematologic malignancy. Treatment focuses on eradicating the underlying plasma cell clone (e.g., chemotherapy, proteasome inhibitors) and managing organ-specific complications. Research continues to explore targeted therapies to inhibit light chain aggregation and improve outcomes.
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