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Basic Information

Enzyme ADAR2
Full Name Adenosine deaminase, RNA specific B1
Location Chr21:45074578..45226563
Ensembl ENSG00000197381
Aliases ADARB1;DRABA2;DRADA2;RED1

Disease Related Information

Disease Species Regulator Expression/Aberrance Target Target Function Regulation Editing-Level Relationship Correlation Editing Effect Phenotype Editing-Dependent PMID
Astrocytomas Human NA Increase GRIA2;GRIK1;GRIK2;BLCAP Receptor for glutamate that functions as a ligand-gated ion channel in the central nervous system and plays an important role in excitatory synaptic transmission. L-glutamate acts as an excitatory neurotransmitter at many synapses in the central nervous system. Deficiency of Glur2 editing may lead to nervous disease. GluR-B Q/R and R/G sites, GluR-6 I/V, Y/C and Q/R sites, GluR-5 Q/R site, Kv1.1 I/V, Gabra-3 I/M site and BLCAP Y/C, Q/R and K/R sites undergo editing events that change amino acid sequence (recoding editing) in crucial positions for protein activity. Increased Causative Negative Compared with the tumor at diagnosis, ADAR2 expression level in the relapse of the case is significantly higher, which can correlate with the rescued editing profiles in the recurrence of this patient. Inhibit tumor progression Yes
Amyotrophic Lateral Sclerosis Human NA Decrease GRIA2 Receptor for glutamate that functions as a ligand-gated ion channel in the central nervous system and plays an important role in excitatory synaptic transmission. L-glutamate acts as an excitatory neurotransmitter at many synapses in the central nervous system. Deficiency of Glur2 editing may lead to nervous disease. Both the appearance of cytoplasmic inclusions containing phosphorylated TAR DNA-binding protein (TDP-43) and inefficient RNA editing at the GluR2 Q/R site are molecular abnormalities observed specifically in motor neurons of patients with sporadic amyotrophic lateral sclerosis (ALS). Decreased Correlated Positive All ADAR2-negative neurons have cytoplasmic inclusions that are immunoreactive to phosphorylated TDP-43, but lacks non-phosphorylated TDP-43 in the nucleus. Promote motor neurons abnormality Yes
Epileptic Mouse NA Decrease GRIA2 Receptor for glutamate that functions as a ligand-gated ion channel in the central nervous system and plays an important role in excitatory synaptic transmission. L-glutamate acts as an excitatory neurotransmitter at many synapses in the central nervous system. Deficiency of Glur2 editing may lead to nervous disease. Reduction in the expression of GluR2 results in an increase in Ca2+-permeable AMPA receptors. Decreased Correlated Positive Defective RNA editing at the GluR2 Q/R site causes neuronal death and that spinal motor neurons are specifically vulnerable to this alteration. Promote motor neurons death Yes
Glaucoma Human NA Decrease GRIA2 Receptor for glutamate that functions as a ligand-gated ion channel in the central nervous system and plays an important role in excitatory synaptic transmission. L-glutamate acts as an excitatory neurotransmitter at many synapses in the central nervous system. Deficiency of Glur2 editing may lead to nervous disease. Chronically elevated IOP reduces expression of the ADAR2 enzyme, which is responsible for GluA2 mRNA Q/R site editing. Decreased Correlated Positive The loss of ADAR2 editing and subsequent disruption of GluA2 RNA editing might potentially play a role in promoting RGC neuronal death as observed in glaucoma. Promote RGC neuronal death in glaucoma Yes
Glioblastoma Human NA Increase GRIA2 Receptor for glutamate that functions as a ligand-gated ion channel in the central nervous system and plays an important role in excitatory synaptic transmission. L-glutamate acts as an excitatory neurotransmitter at many synapses in the central nervous system. Deficiency of Glur2 editing may lead to nervous disease. Overexpression of ADAR2 increases editing of GRIA2 at the Q/R site. Increased Correlated Negative Overexpression of ADAR2 in glioblastoma cell lines slows glioblastoma tumor growth in mice orthotopically injected with glioblastoma cell lines. Inhibit tumor growth Yes
Glioblastoma Human NA Increase miR-222 NA ADAR2 edit miR-222/221 and miR-21 precursors and decrease the expression of the corresponding mature onco-miRNAs. Increased Correlated Negative The rescue of ADAR2 activity decrease the expression of miR-222/221 and inhibit cell proliferation and migration. Inhibit tumor proliferation and migration Yes
ethanol preference Mouse NA Knockout NA NA NA NA Correlation NA NA The nucleus accumbens -specific ADAR2 knockout mice showed a significant decrease in locomotor activity in the open field test although they did not develop anxietyand depression-like behaviors. Yes
systemic lupus erythematosus Human IFNs NA NA NA A to I editing of the 1 site leads to alternative splicing of the ADAR2 pre-mRNA, which in turn results in a non functional enzyme. Increase Correlation Positive Altered editing patterns may be the result of altered expression of ADAR1 and ADAR2 in SLE and type I IFN-activated normal T cells. These altered editing patterns in type I IFN-activated normal T cells may be partially the result of the altered expression of ADAR1p150 and ADAR2. Yes
(C9orf72)amyotrophic lateral sclerosis (ALS) and frontotempo Human NA NA genes of the eiF2 pathway NA NA Increase Correlation Positive the eiF2 pathways�� role in ribosomal function and global protein translation is possibly regulated by ADAR2 the eiF2 pathways role in ribosomal function and global protein translation is possibly regulated by ADAR2 and its dysregulation might contribute to cellular dysfunction in C9orf72 ALS/FTD. Yes