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Rabbit Anti-IFIH1 Antibody (CSB-PA880143ESR2HU)
Supplier: CUSABIO BIOTECH CO.
Clinically amyopathic dermatomyositis autoantigen 140 kDa Antibody,CADM-140 autoantigen Antibody,Helicase with 2 CARD domains Antibody,Helicard Antibody,Interferon-induced with helicase C domain protein 1 Antibody,Melanoma differentiation-associated protein 5 Antibody,MDA-5 Antibody,Murabutide down-regulated protein Antibody,RIG-I-like receptor 2 Antibody,RLR-2 Antibody,RNA helicase-DEAD box protein 116 Antibody,IFIH1 Antibody,MDA5 Antibody, RH116 AntibodyMore alternative names for the antibody
CADM-140 autoantigen antibody|Clinically amyopathic dermatomyositis autoantigen 140 kDa antibody|DEAD/H (Asp Glu Ala Asp/His) box polypeptide antibody|DEAD/H box polypeptide antibody|Helicard antibody|Helicase with 2 CARD domains antibody|Hlcd antibody|IDDM 19 antibody|IDDM19 antibody|IFIH 1 antibody|Ifih1 antibody|IFIH1_HUMAN antibody|Interferon induced helicase C domain containing protein 1 antibody|interferon induced with helicase C domain 1 antibody|Interferon induced with helicase C domain protein 1 antibody|Interferon-induced helicase C domain-containing protein 1 antibody|Interferon-induced with helicase C domain protein 1 antibody|MDA 5 antibody|MDA-5 antibody|Melanoma differentiation associated protein 5 antibody|Melanoma differentiation-associated gene 5 antibody|Melanoma differentiation-associated protein 5 antibody|MGC133047 antibody|Murabutide down regulated protein antibody|Murabutide down-regulated protein antibody|RH 116 antibody|RH116 antibody|RIG I like receptor 2 antibody|RLR 2 antibody|RNA helicase DEAD box protein 116 antibody|RNA helicase-DEAD box protein 116 antibody
Anti-MDA5 antibody (ab69983)
Anti-MDA5 antibody (ab69983)
Recommended applications: ELISA, IHC
Recommended dilution: Recommended dilution:IHC:1:20-1:200
Recommended protocols: check protocols
|Catalogue No.|| |
Recombinant human Interferon-induced helicase C domain-containing protein 1 protein (1-205AA)
|Recommended dilution|| |
|Molecular weight|| |
Antigen Affinity Purified
Shipped at 4 Celcius Degree. Upon delivery aliquot and store at -20 Celcius Degree or -80 Celcius Degree. Avoid repeated freeze.
|Protein function|| |
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). 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. .
|Protein tissue specificity|| |
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. .
|Involvement in disease|| |
Diabetes mellitus, insulin-dependent, 19 (IDDM19) [MIM:610155]: A multifactorial disorder of glucose homeostasis that is characterized by susceptibility to ketoacidosis in the absence of insulin therapy. Clinical features are polydipsia, polyphagia and polyuria which result from hyperglycemia-induced osmotic diuresis and secondary thirst. These derangements result in long-term complications that affect the eyes, kidneys, nerves, and blood vessels. . Note=Disease susceptibility may be associated with variations affecting the gene represented in this entry.; Note=IFIH1 is the CADM-140 autoantigen, involved in clinically amyopathic dermatomyositis (CADM). This is a chronic inflammatory disorder that shows typical skin manifestations of dermatomyositis but has no or little evidence of clinical myositis. Anti-CADM-140 antibodies appear to be specific to dermatomyositis, especially CADM. Patients with anti-CADM-140 antibodies frequently develop life-threatening acute progressive interstitial lung disease (ILD). .; Aicardi-Goutieres syndrome 7 (AGS7) [MIM:615846]: A form of Aicardi-Goutieres syndrome, a genetically heterogeneous disease characterized by cerebral atrophy, leukoencephalopathy, intracranial calcifications, chronic cerebrospinal fluid (CSF) lymphocytosis, increased CSF alpha-interferon, and negative serologic investigations for common prenatal infection. Clinical features as thrombocytopenia, hepatosplenomegaly and elevated hepatic transaminases along with intermittent fever may erroneously suggest an infective process. Severe neurological dysfunctions manifest in infancy as progressive microcephaly, spasticity, dystonic posturing and profound psychomotor retardation. Death often occurs in early childhood. . Note=The disease is caused by mutations affecting the gene represented in this entry.; Singleton-Merten syndrome 1 (SGMRT1) [MIM:182250]: An autosomal dominant disorder with variable expression. Core features are marked aortic calcification, dental anomalies, osteopenia, acro-osteolysis, and to a lesser extend glaucoma, psoriasis, muscle weakness, and joint laxity. Dental anomalies include delayed eruption and immature root formation of anterior permanent teeth, early loss of permanent teeth due to short roots, acute root resorption, high caries, and aggressive alveolar bone loss. Additional clinical manifestations include particular facial characteristics and abnormal joint and muscle ligaments. . Note=The disease is caused by mutations affecting the gene represented in this entry.
|Protein sequence and domain|| |
Belongs to the helicase family. RLR subfamily.
|Protein post-translational modifications|| |
Sumoylated. Sumoylation positively regulates its role in type I interferon induction and is enhanced by PIAS2-beta. .; Ubiquitinated by RNF125, leading to its degradation by the proteasome (PubMed:17460044). USP17/UPS17L2-dependent deubiquitination positively regulates the receptor (PubMed:20368735). .; During apoptosis, processed into 3 cleavage products. The helicase-containing fragment, once liberated from the CARD domains, translocate from the cytoplasm to the nucleus. The processed protein significantly sensitizes cells to DNA degradation. .
|Protein cellular localization|| |
Cytoplasm . Nucleus . Note=May be found in the nucleus, during apoptosis.
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