IFIH1 Recombinant Monoclonal Antibody
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中文名稱：IFIH1重組抗體
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貨號：CSB-RA963423A0HU
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規格：￥1320
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圖片：
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Western Blot
Positive WB detected in: THP-1 whole cell lysate, Mouse lung tissue
All lanes: MDA5 antibody at 1:2000
Secondary
Goat polyclonal to rabbit IgG at 1/50000 dilution
Predicted band size: 117, 26 kDa
Observed band size: 117 kDa
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其他：
產品詳情
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產品描述：CSB-RA963423A0HU 重組單克隆抗體是針對IFIH1/MDA5靶點研發的高特異性檢測試劑。該抗體靶向的IFIH1（干擾素誘導解旋酶C結構域蛋白1）是固有免疫系統的關鍵模式識別受體，通過識別病毒雙鏈RNA激活MAVS信號通路，在抗病毒免疫應答和自身免疫性疾病發生中發揮重要作用。經嚴格驗證，本產品在Western Blot實驗中能特異性識別天然及重組表達的IFIH1蛋白，推薦使用濃度范圍為1:500至1:5000，可呈現清晰單一的目標條帶，且在ELISA檢測體系中展現優異的抗原結合能力。抗體生產采用重組表達技術，確保批次間高度一致性，適用于病毒感染機制研究、自身免疫性疾病（如Aicardi-Goutières綜合征、皮肌炎）相關信號通路探索、先天免疫調控機理分析等科研領域，為解析IFIH1在病原體識別及炎癥反應中的生物學功能提供可靠工具。
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Uniprot No.：
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基因名：
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別名：Interferon-induced helicase C domain-containing protein 1 (EC 3.6.4.13) (Clinically amyopathic dermatomyositis autoantigen 140 kDa) (CADM-140 autoantigen) (Helicase with 2 CARD domains) (Helicard) (Interferon-induced with helicase C domain protein 1) (Melanoma differentiation-associated protein 5) (MDA-5) (Murabutide down-regulated protein) (RIG-I-like receptor 2) (RLR-2) (RNA helicase-DEAD box protein 116), IFIH1, MDA5 RH116
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反應種屬：Human, Mouse
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免疫原：A synthesized peptide derived from human MDA5
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免疫原種屬：Homo sapiens (Human)
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標記方式：Non-conjugated
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克隆類型：Monoclonal
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抗體亞型：Rabbit IgG
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純化方式：Affinity-chromatography
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克隆號：3D12
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：Rabbit IgG in 10mM phosphate buffered saline , pH 7.4, 150mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.
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產品提供形式：Liquid
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應用范圍：ELISA, WB
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推薦稀釋比：
Application Recommended Dilution WB 1:500-1:5000 -
Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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貨期：Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
相關產品
靶點詳情
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功能：Innate immune receptor which acts as a cytoplasmic sensor of viral nucleic acids and plays a major role in sensing viral infection and in the activation of a cascade of antiviral responses including the induction of type I interferons and proinflammatory cytokines. Its ligands include mRNA lacking 2'-O-methylation at their 5' cap and long-dsRNA (>1 kb in length). Upon ligand binding it associates with mitochondria antiviral signaling protein (MAVS/IPS1) which activates the IKK-related kinases: TBK1 and IKBKE which phosphorylate interferon regulatory factors: IRF3 and IRF7 which in turn activate transcription of antiviral immunological genes, including interferons (IFNs); IFN-alpha and IFN-beta. Responsible for detecting the Picornaviridae family members such as encephalomyocarditis virus (EMCV) and mengo encephalomyocarditis virus (ENMG). Detects coronavirus SARS-CoV-2. Can also detect other viruses such as dengue virus (DENV), west Nile virus (WNV), and reovirus. Also involved in antiviral signaling in response to viruses containing a dsDNA genome, such as vaccinia virus. Plays an important role in amplifying innate immune signaling through recognition of RNA metabolites that are produced during virus infection by ribonuclease L (RNase L). May play an important role in enhancing natural killer cell function and may be involved in growth inhibition and apoptosis in several tumor cell lines.
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基因功能參考文獻：
- RIG-I and MDA5 receptor sensing of host non-coding RNAs facilitates the cell-intrinsic immune response to Kaposi's sarcoma-associated herpesvirus infection. PMID: 30451863
- Melanoma differentiation-associated gene 5 (MDA5) is a recently described autoantigen target in a subset of patients with dermatomyositis. Anti-MDA5 dermatomyositis is characterized by a unique mucocutaneous and systemic phenotype PMID: 29229575
- genetic association studies in population of children in Hungary: Data suggest that a genetic polymorphism in IFIH1 (rs1990760) is associated with seasonal variation in onset of type 1 diabetes in population studied; this polymorphism in IFIH1 may contribute to manifestation of type 1 diabetes primarily in summer. PMID: 28929635
- Anti-MDA5 antibody helps in the diagnosis of dermatomyositis, predicting cutaneous, musculoskeletal and pulmonary manifestations. PMID: 29380533
- Engineered Oncolytic Poliovirus PVSRIPO Subverts MDA5-Dependent Innate Immune Responses in Cancer Cells. PMID: 29997212
- This work validates the significant role of MDA5 in IFN signaling and HCV infection and proposes the first lncRNA inhibiting HCV replication by promoting the activation of MDA5 and mediating the association between MDA5 and HCV RNA, the study of which may shed light on the MDA5 function and treatment for hepatitis C patients. PMID: 29899107
- anti-MDA5 antibody related to muscle weakness, Gottron's sign or papules, mechanic's hand, V rash, skin ulcers, panniculitis, alopecia, arthritis/arthralgia, pneumomediastinum, and elevated CK in patients with dermatomyositis [meta-analysis] PMID: 28983955
- the identification of a pathogen-restricted immunodeficiency due to loss-of-function variants in IFIH1, which result in defective innate recognition of RNA viruses, preventing the activation of an efficient antiviral IFN response. PMID: 28716935
- The connection between the rs1990760 polymorphism and the expression level of type III IFNs. PMID: 28000722
- Oncostatin M induces RIG-I and MDA5 expression and enhances the double-stranded RNA response in fibroblasts. PMID: 28560754
- data highlight new function of ARRDC4 in innate immunity, contributing to the better understanding about regulation of MDA5 activation after EV71 infection, and also suggest ARRDC4 may serve as a potential target for intervention of EV71-induced inflammatory response. PMID: 28594402
- Both diseases (Neuroinflammation and the Singleton-Merten Syndrome) are part of the interferonopathy grouping and that the Arg822Gln mutation in IFIH1 can cause a spectrum of disease, including neurological involvement. PMID: 28475458
- Knockdown of PBRM1 in colon cancer cells increased the expression of two receptor genes (RIG-I and MDA5) and upregulated interferon (IFN)-related and inflammation-related gene signatures. PMID: 28940253
- Dengue virus infection of human dendritic cells drives follicular T helper cells formation via crosstalk of RIG-I and MDA5. PMID: 29186193
- study revealed that encapsidation of DI-RNA (defective interfering genomes) molecules within the measles virus nucleocapsid abolished their immunoactive properties. identified specific interactions of DI-RNAs with both RIG-I and LGP2 but not MDA5. PMID: 28768856
- An up-regulation of type I IFN-induced gene transcripts in all 5 patients tested and a heterozygous gain-of-function mutation in IFIH1 in each family were discovered in families with a Singleton-Merten syndrome phenotype. PMID: 28605144
- Data indicate autosomal recessive, homozygous MDA5 encoding gene IFIH1 mutation in the proband, and both parents and a brother were carriers of this mutation. PMID: 28606988
- this study shows that PACT facilitates RNA-induced formation of MDA5 oligomers in response to viruses PMID: 28760879
- Human peripheral blood mononuclear cells (PBMCs) and cell lines expressing the risk variant IFIH1(T946) exhibited heightened basal and ligand-triggered production of type I interferons. PMID: 28553952
- Mechanistically, West Nile virus NS1 targets RIG-I and melanoma differentiation-associated gene 5 (MDA5) by interacting with them and subsequently causing their degradation by the proteasome. PMID: 28659477
- The rare variant rs35667974, encoding an Ile923Val amino acid change in the IFIH1 gene protein product, is protective for psoriatic arthritis. PMID: 28501801
- This would suggest that the change in IP-10 is genotypically driven, while the change in IL-6 may be reflective of systemic lupus erythematosus (SLE) transition status. These data suggest that IFIH1 may contribute to SLE pathogenesis via altered inflammatory mechanisms. PMID: 28234905
- dynamic sumoylation and desumoylation of MDA5 and RIG-I modulate efficient innate immunity to RNA virus and its timely termination. PMID: 28250012
- GPATCH3 negatively regulates both RIG-I- and MDA5-mediated signaling. PMID: 28414768
- Data support a new model where an LGP2-MDA5 oligomer shuttles NS3 to the mitochondria to block antiviral signaling PMID: 28483922
- MDA-5 stimulation leads to endothelial dysfunction. PMID: 27130701
- LGP2 plays an essential role in activating interferon signaling against hepatitis C virus (HCV) infection by promoting MDA5 recognition of HCV pathogen-associated molecular patterns. PMID: 28090671
- These data confirm the pathogenicity of IFIH1 c.2465G>A p.(Arg822Gln) for Singleton-Merten syndrome and affirm the striking phenotypic heterogeneity of this disorder PMID: 28319323
- RNAs isolated from HCV-replicating cells triggered robust IFN-beta and IFN-lambda production through MDA5. PMID: 27655134
- meta-analysis of peer-reviewed articles from 2008 to 2015 and data from this study indicated an association between rs1990760 and Systemic lupus erythematosus (SLE) onset . This is the first association analysis on IFIH1 polymorphisms and SLE susceptibility in Brazilian populations. PMID: 27813554
- This study reviewed that Neurologic Phenotypes Associated with Mutations in IFIH1 in patients with Aicardi-Goutieres Syndrome. PMID: 27643693
- We provide herein the results of pathological examination including detailed description of the neuropathological hallmarks. To our knowledge, this the first detailed clinico-pathological description of a patient with an IFIH1 pathogenic variant. PMID: 26833990
- Our findings indicate that although STAT4 and IFIH1 SNPs are not associated with T1D in a Brazilian population, they might play a role in susceptibility to T1D on a larger worldwide scale PMID: 26782418
- Results describe a family with gain of function mutation associated with cutaneous manifestations of Aicardi-Goutieres and Singleton-Merten syndromes PMID: 26284909
- These results showed that mRNA levels of MDA5 and RIG-1 were significantly decreased and increased, respectively, in chronic hepatitis B patients when compared to healthy controls. PMID: 26485346
- Findings indicate that melanoma differentiation associated protein-5 (MDA5) may serve as a complementary role in the toll-like receptor3 (TLR3) activated suppression of neuroblastoma (NB). PMID: 26208481
- The MDA5 knockdown had no significant impact on the phosphorylation of eukaryotic translation initiation factor 2alpha triggered by CCCP, and MDA5 itself was not recruited to SGs, and the regulation of MDA5 in the SG response occurs downstream of eIF2alpha. PMID: 26351918
- A significantly higher frequency of anti-melanoma differentiation-associated gene 5 (MDA5) antibodies was observed in Chinese patients with polymyositis/dermatomyositis (PM/DM) than in Japanese patients. PMID: 25903820
- MDA5 mediates neuroprotection against cerebral ischemic injury. PMID: 26564103
- discusses the different risk and protective IFIH1 alleles in the context of recent structural and functional analysis that relate to MDA5 regulation of interferon responses PMID: 26385483
- The study proposes that the turnover kinetics of the ATPase domain enables the discrimination of self/non-self RNA by both RIG-I and MDA5. PMID: 26215161
- This review briefly summarizes the RLR system, and focuses on the relationship between LGP2 and MDA5, describing in detail how these two proteins work together to detect foreign RNA and generate a fully functional antiviral response. PMID: 25794939
- Single nucleotide polymorphism in IFIH1 gene is associated with type 1 diabetes. PMID: 25042601
- the ectopic expression of RIOK3 or a phosphomimetic MDA5-S828D mutation attenuated MDA5-mediated signaling. PMID: 25865883
- As a receptor for viral RNA, MDA5 until now has been linked to antiviral host defense, but these novel studies show unexpected effects in antifungal immunity as well. PMID: 25579795
- MDA5 plays important role in sensing HCV infection to trigger interferon response. PMID: 25463548
- L region antisense RNA of EMCV is a key determinant of innate immunity to the virus and represents an RNA that activates LGP2 associated MDA5 in virally-infected cells. PMID: 24550253
- These data demonstrate that EFTUD2 restricts Hepatitis C Virus infection mainly through an RIG-I/MDA5-mediated, JAK-STAT-independent pathway, thereby revealing the participation of EFTUD2 as a novel innate immune regulator. PMID: 25878102
- There is an association of the IFIH1 locus with susceptibility to type 1 diabetes mellitus in the Polish population. PMID: 25515714
- Data indicate that pattern recognition receptors RIG-I and MDA5 displace viral proteins bound to dsRNA in an ATP-dependent manner. PMID: 25891073
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相關疾病：Diabetes mellitus, insulin-dependent, 19 (IDDM19); Aicardi-Goutieres syndrome 7 (AGS7); Singleton-Merten syndrome 1 (SGMRT1)
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亞細胞定位：Cytoplasm. Nucleus. Mitochondrion. Note=Upon viral RNA stimulation and ISGylation, translocates from cytosol to mitochondrion. May be found in the nucleus, during apoptosis.
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蛋白家族：Helicase family, RLR subfamily
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組織特異性：Widely expressed, at a low level. Expression is detected at slightly highest levels in placenta, pancreas and spleen and at barely levels in detectable brain, testis and lung.
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數據庫鏈接：
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