| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 1/100-1/200 | Human,Mouse,Rat |
| IHC | 1/100-1/200 | 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 |
| Host/Isotype | Mouse IgG2a |
| 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 | Purified recombinant fragment of human IFIH1 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide |
+ +
以下是关于IFIH1抗体的3篇参考文献，涵盖其在不同疾病中的作用和研究进展：
---
1. **文献名称**：*Anti-IFIH1 Antibodies in Dermatomyositis: A Marker of Clinical Heterogeneity*
**作者**：Bursztejn AC, et al.
**摘要**：该研究报道了抗IFIH1(MDA5)抗体在皮肌炎患者中的存在，并揭示其与特定临床表型(如快速进展性间质性肺病)的关联。研究强调此类抗体可作为疾病分型和预后的生物标志物。
---
2. **文献名称**：*Gain-of-Function Mutations in IFIH1 Cause a Spectrum of Human Disease Phenotypes Associated with Upregulated Type I Interferon Signaling*
**作者**：Rice GI, et al.
**摘要**：文章探讨了IFIH1功能获得性突变导致I型干扰素通路过度激活的机制，涉及Aicardi-Goutières综合征(AGS)和单基因狼疮等自身免疫性疾病。部分研究指出患者体内可能产生针对MDA5的自身抗体，加剧炎症反应。
---
3. **文献名称**：*Autoantibodies Against MDA5 and IFIH1 in Patients with Inflammatory Myopathies*
**作者**：Betteridge Z, et al.
**摘要**：该研究分析了炎症性肌病患者血清中的抗MDA5(IFIH1编码)抗体，发现其与无肌病性皮肌炎及严重皮肤溃疡相关，提示抗体可能通过干扰素通路调控疾病进程。
---
如需更具体内容，可进一步提供研究方向或疾病背景以便筛选文献。
The IFIH1 gene encodes MDA5 (Melanoma Differentiation-Associated protein 5), a cytoplasmic RNA helicase and key component of the innate immune system. As a pattern recognition receptor, MDA5 detects viral double-stranded RNA, triggering interferon (IFN) production and antiviral responses. Dysregulation of IFIH1 is linked to autoimmune and inflammatory diseases. Gain-of-function mutations in IFIH1 cause Aicardi-Goutières syndrome (AGS), a severe neuroinflammatory disorder, while certain polymorphisms are associated with type 1 diabetes and systemic lupus erythematosus.
Autoantibodies against MDA5 (anti-MDA5) are clinically significant biomarkers, particularly in dermatomyositis (DM). These antibodies are strongly associated with clinically amyopathic DM and a high risk of rapidly progressive interstitial lung disease (RP-ILD), which carries poor prognosis. Detection of anti-MDA5 antibodies via ELISA or immunoprecipitation aids in diagnosis, risk stratification, and monitoring disease progression.
Research suggests MDA5 activation drives pathologic IFN signaling, making it a potential therapeutic target. JAK inhibitors and other IFN pathway modulators are being explored. Conversely, loss of MDA5 function increases susceptibility to viral infections. IFIH1 antibodies thus serve dual roles: as diagnostic tools in autoimmunity and as functional probes to study antiviral immunity. Their detection underscores the interplay between innate immunity and autoinflammation in human disease.
×
