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Adenosine deaminase tRNA-specific 3 (ADAT3) is a non-catalytic regulatory subunit of a heterodimeric enzyme complex (with ADAT2) responsible for modification of transfer RNA (tRNA) molecules, specifically converting adenosine to inosine at the wobble position (position 34) of tRNA anticodons[1][3][5][8]. This RNA modification is essential for precise decoding of genetic information during protein synthesis. Although ADAT3 does not possess catalytic activity, it facilitates proper substrate recognition, tRNA binding, and positioning, thereby enabling ADAT2 to perform the deamination reaction[3][4]. Mutations in ADAT3 disrupt tRNA editing, leading to autosomal recessive neurodevelopmental disorders characterized by intellectual disability, brain abnormalities, poor growth, and various dysmorphic features[1][3][6][9]. No approved drugs are known to target ADAT3 directly, and its main clinical relevance arises from the pathogenesis of rare genetic disorders rather than as a therapeutic target or receptor.
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