英文名称
IFIH1 Recombinant Rabbit mAb
英文别名
AGS7; Hlcd; IDDM19; IMD95; MDA-5; MDA5; RLR-2; SGMRT1; 9130009C22Rik; Helicard; IFIH1_HUMAN; IFIH1; 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; 3.6.4.13; RH116; IFIH1_MOUSE; Interferon induced with helicase C domain protein 1; interferon induced with helicase C domain 1; melanoma differentiation-associated gene 5
免疫原
A synthesized peptide derived from human MDA5: 950-1025
纯化方法
affinity purified by Protein A
储存液
10mM phosphate buffered saline(pH 7.4) with 150mM sodium chloride, 0.05% BSA, 0.02% Proclin300 and 50% glycerol.
保存条件
Store at 4℃ for short term. Store at -20℃ for long term. Avoid repeated freeze/thaw cycles.
注意事项
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
背景资料
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 pro-inflammatory cytokines
蛋白名
Interferon-induced helicase C domain-containing protein 1
亚细胞定位
Cytoplasm. Nucleus. May be found in the nucleus, during apoptosis.
组织特异性
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.
翻译后修饰
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.
疾病
Genetic variation in IFIH1 is associated with diabetes mellitus insulin-dependent type 19 (IDDM19) [MIM:610155]. A multifactorial disorder of glucose homeostasis that is characterized by susceptibility to ketoacidosis in the absence of insulin therapy. Clinical fetaures 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=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).
相似性
Belongs to the helicase family.
Contains 2 CARD domains.
Contains 1 helicase ATP-binding domain.
Contains 1 helicase C-terminal domain.
功能
RNA helicase that, through its ATP-dependent unwinding of RNA, may function to promote message degradation by specific RNases. Seems to have growth suppressive properties. Involved in innate immune defense against viruses. Upon interaction with intracellular dsRNA produced during viral replication, triggers a transduction cascade involving MAVS/IPS1, which results in the activation of NF-kappa-B, IRF3 and IRF7 and the induction of the expression of antiviral cytokines such as IFN-beta and RANTES (CCL5). ATPase activity is specifically induced by dsRNA. Essential for the production of interferons in response to picornaviruses.