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Rabbit Anti-IFIH1  antibody (bs-18740R)  
~~~促銷代碼KT202411~~~
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產品編號 bs-18740R
英文名稱 Rabbit Anti-IFIH1  antibody
中文名稱 黑色素瘤分化相關蛋白5抗體
別    名 CADM-140 autoantigen; Clinically amyopathic dermatomyositis autoantigen 140 kDa; DEAD/H (Asp Glu Ala Asp/His) box polypeptide; DEAD/H box polypeptide; Helicard; Helicase with 2 CARD domains; Hlcd; IDDM 19; IDDM19; IFIH 1; IFIH1_HUMAN; Interferon induced helicase C domain containing protein 1; interferon induced with helicase C domain 1; Interferon induced with helicase C domain protein 1; Interferon-induced helicase C domain-containing protein 1; Interferon-induced with helicase C domain protein 1; MDA 5; MDA-5; MDA5; Melanoma differentiation associated protein 5; Melanoma differentiation-associated gene 5; Melanoma differentiation-associated protein 5; MGC133047; Murabutide down regulated protein; Murabutide down-regulated protein; RH 116; RH116; RIG I like receptor 2; RLR 2; RNA helicase DEAD box protein 116; RNA helicase-DEAD box protein 116.  
研究領域 細胞生物  糖尿病  細胞分化  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human,Mouse,Rat,Rabbit,Pig,Sheep,Cow,Horse)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 117kDa
細胞定位 細胞核 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human IFIH1: 901-1025/1025 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 DEAD box proteins, characterized by the conserved motif Asp-Glu-Ala-Asp (DEAD), are putative RNA helicases. They are implicated in a number of cellular processes involving alteration of RNA secondary structure such as translation initiation, nuclear and mitochondrial splicing, and ribosome and spliceosome assembly. Based on their distribution patterns, some members of this family are believed to be involved in embryogenesis, spermatogenesis, and cellular growth and division. This gene encodes a DEAD box protein that is upregulated in response to treatment with beta-interferon and a protein kinase C-activating compound, mezerein. Irreversible reprogramming of melanomas can be achieved by treatment with both these agents; treatment with either agent alone only achieves reversible differentiation. Genetic variation in this gene is associated with diabetes mellitus insulin-dependent type 19. [provided by RefSeq, Jul 2012]

Function:
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.

Subcellular Location:
Cytoplasm. Nucleus. May be found in the nucleus, during apoptosis.

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.

Post-translational modifications:
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.

DISEASE:
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).

Similarity:
Belongs to the helicase family.
Contains 2 CARD domains.
Contains 1 helicase ATP-binding domain.
Contains 1 helicase C-terminal domain.

SWISS:
Q9BYX4

Gene ID:
64135

Database links:

Entrez Gene: 64135 Human

Entrez Gene: 71586 Mouse

Omim: 606951 Human

SwissProt: Q9BYX4 Human

SwissProt: Q8R5F7 Mouse

Unigene: 163173 Human

Unigene: 136224 Mouse



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