Names & Taxonomy

Uniprot ID:
Q9BYX4
Entry Name:
IFIH1_HUMAN
Status:
reviewed
Protein Names:
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)
Gene Names:
IFIH1 MDA5 RH116
Gene Names Primary:
IFIH1
Organism:
Homo sapiens (Human)

Structure

Length:
1025
Sequence:
MSNGYSTDENFRYLISCFRARVKMYIQVEPVLDYLTFLPAEVKEQIQRTVATSGNMQAVELLLSTLEKGVWHLGWTREFVEALRRTGSPLAARYMNPELTDLPSPSFENAHDEYLQLLNLLQPTLVDKLLVRDVLDKCMEEELLTIEDRNRIAAAENNGNESGVRELLKRIVQKENWFSAFLNVLRQTGNNELVQELTGSDCSESNAEIENLSQVDGPQVEEQLLSTTVQPNLEKEVWGMENNSSESSFADSSVVSESDTSLAEGSVSCLDESLGHNSNMGSDSGTMGSDSDEENVAARASPEPELQLRPYQMEVAQPALEGKNIIICLPTGSGKTRVAVYIAKDHLDKKKKASEPGKVIVLVNKVLLVEQLFRKEFQPFLKKWYRVIGLSGDTQLKISFPEVVKSCDIIISTAQILENSLLNLENGEDAGVQLSDFSLIIIDECHHTNKEAVYNNIMRHYLMQKLKNNRLKKENKPVIPLPQILGLTASPGVGGATKQAKAEEHILKLCANLDAFTIKTVKENLDQLKNQIQEPCKKFAIADATREDPFKEKLLEIMTRIQTYCQMSPMSDFGTQPYEQWAIQMEKKAAKEGNRKERVCAEHLRKYNEALQINDTIRMIDAYTHLETFYNEEKDKKFAVIEDDSDEGGDDEYCDGDEDEDDLKKPLKLDETDRFLMTLFFENNKMLKRLAENPEYENEKLTKLRNTIMEQYTRTEESARGIIFTKTRQSAYALSQWITENEKFAEVGVKAHHLIGAGHSSEFKPMTQNEQKEVISKFRTGKINLLIATTVAEEGLDIKECNIVIRYGLVTNEIAMVQARGRARADESTYVLVAHSGSGVIEHETVNDFREKMMYKAIHCVQNMKPEEYAHKILELQMQSIMEKKMKTKRNIAKHYKNNPSLITFLCKNCSVLACSGEDIHVIEKMHHVNMTPEFKELYIVRENKALQKKCADYQINGEIICKCGQAWGTMMVHKGLDLPCLKIRNFVVVFKNNSTKKQYKKWVELPITFPNLDYSECCLFSDED
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Cytoplasm

Function

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.
Catalytic Activity:
ATP + H(2)O = ADP + phosphate.
Gene Ontology Go:
cytosol
nucleus
ATP binding
DNA binding
double-stranded RNA binding
helicase activity
ribonucleoprotein complex binding
single-stranded RNA binding
zinc ion binding
cytoplasmic pattern recognition receptor signaling pathway in response to virus
detection of virus
innate immune response
MDA-5 signaling pathway
negative regulation of type I interferon production
positive regulation of interferon-alpha production
positive regulation of interferon-beta production
protein sumoylation
regulation of type III interferon production
response to virus
viral process
Gene Ontology Biological Process:
cytoplasmic pattern recognition receptor signaling pathway in response to virus
detection of virus
innate immune response
MDA-5 signaling pathway
negative regulation of type I interferon production
positive regulation of interferon-alpha production
positive regulation of interferon-beta production
protein sumoylation
regulation of type III interferon production
response to virus
viral process
Gene Ontology Molecular Function:
ATP binding
DNA binding
double-stranded RNA binding
helicase activity
ribonucleoprotein complex binding
single-stranded RNA binding
zinc ion binding
Gene Ontology Cellular Component:
cytosol
nucleus
Keywords:
3D-structure
ATP-binding
Aicardi-Goutieres syndrome
Alternative splicing
Antiviral defense
Complete proteome
Cytoplasm
Diabetes mellitus
Disease mutation
Helicase
Host-virus interaction
Hydrolase
Immunity
Innate immunity
Metal-binding
Nucleotide-binding
Nucleus
Phosphoprotein
Polymorphism
RNA-binding
Reference proteome
Repeat
Ubl conjugation
Zinc
Interacts With:
Q7Z434; P11207; P30927; Q9EMA9; P04487

Publication

PubMed ID:
11805321 14645903 15489334 14702039 12015121 15563593 16127453 16177806 17600090 19565506 19211564 19656871 19881509 20434986 20368735 20015976 20068231 21269460 21616437 21884169 21742966 20950133 21791617 21156324 21245900 21217758 22160685 22301138 23711367 24275569 16699517 24995871 24686847 25620204 19531363 19380577