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Adenosine deaminase tRNA-specific 3 (ADAT3)

Target
ADAT3
Molecular classification
Enzyme (non-catalytic subunit of tRNA-specific adenosine deaminase complex), Other (regulatory/structural protein in RNA modification complex)
01

Overview

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.

Other names
Probable inactive tRNA-specific adenosine deaminase-like protein 3TAD3tRNA-specific adenosine-34 deaminase subunit ADAT3Adenosine deaminase, tRNA-specific 3, TAD3 homolog (S. cerevisiae)tRNA-specific adenosine deaminase 3 homolog (S. cerevisiae)MRT36FWP005S863-5
02

Biological functions

tRNA modification (adenosine to inosine conversion at tRNA wobble position)Regulation of translation accuracy via tRNA anticodon editingStructural/support role in enzyme complex formation
03

Disease associations

Neurodevelopmental disorder with brain abnormalities, poor growth, and dysmorphic faciesIntellectual disabilityEsotropia (crossed eyes)
04

Safety considerations

Neurodevelopmental and growth impairment due to ADAT3 loss-of-function mutationsPotential impact on protein synthesis fidelity if ADAT3 is dysfunctional
05

Biomarkers

ADAT3 genetic mutations as biomarkers for neurodevelopmental syndromes

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