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Symbol ADAR contributors: mct/npt/pgu - updated : 28-03-2015
HGNC name adenosine deaminase, RNA-specific
HGNC id 225
Corresponding disease
AGS6 Aicardi-Goutičres syndrome 6
DSH1 dyschromatosis symmetrica hereditaria 1
Location 1q21.3      Physical location : 154.554.535 - 154.600.456
Synonym name
  • double-stranded RNA adenosine editing deaminase
  • 136 kDa double-stranded RNA binding protein
  • interferon-inducible protein 4
  • Synonym symbol(s) DRADA, DSRAD, ADAR1, DSH, IFI4, K88dsRBP, p136, G1P1
    EC.number 3.5.4.-
    TYPE functioning gene
    STRUCTURE 45.90 kb     15 Exon(s)
    Genomic sequence alignment details
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status provisional
    Map cen - D6S1816E - D6S2591 - ADAR - D6S2017 - D6S1794E - qter
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    15 initiation site 6640 136 1226 ubiquitous 2011 21316340
  • ADAR-p150 or variant 1, using exon 1A, ADAR-A
  • interferon inducible and shuttling between the nucleus and cytoplasm
  • three double-stranded (ds)RNA-binding domains and the deaminase domain
  • N-terminally extended compared to p110, and possesses two copies of a Z-DNA binging motif
  • 15 splicing 6532 110 931 constitutively expressed, ubiquitous 2011 21316340
  • ADAR-p110 or variant 4 using exon 1B
  • three double-stranded (ds)RNA-binding domains and the deaminase domain
  • lacks the Z-DNA binding domains that are present in ADAR-p150
  • high expression of p110 was detected in leukemias, especially in B-ALL, but decrease of p110 expression was observed in B-ALL patients achieving complete remission
  • 15 splicing 6562 - 1200 ubiquitous 2008 18835380
  • ADAR-b or variant 2
  • deletion within exon 7
  • 15 splicing 6505 - 1181 ubiquitous 2008 18835380
  • ADAR-c or variant 3
  • deletion within exon 7 and 6
  • 15 - 6730 - 931 - 2011 21316340
    Type ubiquitous
       expressed in (based on citations)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Nervousbrain   highly
    Reproductivemale systemtestis    Mus musculus
    Respiratorylung   highly
    SystemCellPubmedSpeciesStageRna symbol
    ReproductiveSertoli cell Mus musculus
    cell lineage
    cell lines
    at STAGE
  • one or two N-terminal Z-DNA–binding domains, two motifs Z alpha and Z beta acting as a single bipartite left handed Z-DNA binding domain (involved in the resistance to proteases)
  • three centrally located dsRNA-binding domains (DRBM)
  • nuclear localization signal at the C terminus (NLS-c), consisting of a bipartite basic amino acid motif plus the last 39 residues regulating the nuclear import
  • C-terminal deaminase catalytic domain
  • mono polymer monomer
    interspecies homolog to murine Adar
  • ADAR gene family
  • CATEGORY enzyme , RNA associated
    SUBCELLULAR LOCALIZATION     intracellular
    text deletion of the NLS-c causes the truncated ADAR1 protein to be retained in the cytoplasm
    basic FUNCTION
  • double-stranded RNA adenosine editing deaminase
  • involved in site-selective editing of neural glutamate and serotonin receptors and in generation of biased hypermutations of viral DNA
  • required for embryonic erythropoiesis in mice
  • plays an essential role in adult hematopoiesis through its RNA editing activity in hematopoietic stem cells
  • novel regulatory role for ADAR activation under inflammatory stress to both promote muscle protein synthesis pathways and limit atrophy pathways
  • ADAR functioned to promote survival of numerous tissues by editing one or more double-stranded RNAs required for protection against stress-induced apoptosis
  • dysfunction of ADAR might be an important event in leukemias
  • ADARB1 and ADAR have evolved highly conserved, distinct functions
  • functions as a suppressor of dsRNA-mediated antiviral responses including activation of EIF2AK2 and interferon regulatory factor 3
  • possible role for ADAR as a suppressor of type I interferon signaling
  • may limit the cytoplasmic accumulation of the dsRNA generated from genomic repetitive elements
  • having potentially a role in the metabolism of retroelements and a major contribution of ADAR to evolutionary fitness may be to regulate the accumulation of dsRNA generated from basally transcribed repetitive sequences within the genome
  • catalyze the hydrolytic deamination of adenosine to inosine in double-stranded RNA (dsRNA) and thereby potentially alter the information content and structure of cellular RNAs
  • regulates osteoblast differentiation by, at least in part, modulation of osterix expression, which is essential for bone formation
  • promotes malignant progenitor reprogramming in chronic myeloid leukemia
  • plays a major role in splicing regulation
  • ADAR coordinates with ADARB1 to regulate editing and stability of Cat2 transcribed nuclear RNA (Ctn RNA)
  • CELLULAR PROCESS protein, editing
    a component
  • component of large nuclear ribonucleoproteins particles, associated with splicing factors
  • endogenous ADAR can form heterodimers with ADARB1 in astrocytes
    small molecule
  • large nuclear ribonucleoproteins
  • interacting with ILF3 (can regulate transcription by interaction with ILF3)
  • is a molecular target of BCL6 (BCL6 protects senescent cells from accumulation of adenosine-targeted DNA mutations induced by ADAR)
  • ADAR, but not ADAR2, takes part in the editing of F11R however editing alone is not sufficient for obtaining an elevation in RNA levels
  • cell & other
    induced by interferon
    induced during acute inflammation
    corresponding disease(s) DSH1 , AGS6
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral     --over  
    highly expressed in high grade astrocytomas
    Variant & Polymorphism
    Candidate gene
  • p110 expression level might be a potential clinical marker monitoring chemotherapy in pediatric B-ALL
  • Therapy target