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TitleNeurodevelopmental disorder-linked Argonaute mutations permit delayed RISC formation and unusual shortening of miRNAs by 3'→5' trimming.
Journal, issue, pagesProc Natl Acad Sci U S A, Vol. 122, Issue 46, Page e2524644122, Year 2025
Publish dateNov 18, 2025
AuthorsAndrew Savidge / Huaqun Zhang / Vishal Annasaheb Adhav / Audrey C Kehling / GeunYoung Sim / Zhangfei Shen / Tian-Min Fu / Kotaro Nakanishi /
PubMed AbstractMutations in Argonaute proteins (AGOs) cause Argonaute syndrome, a neurodevelopmental disorder (NDD), yet the underlying molecular mechanism remains unclear. We determined cryo-electron microscopy ...Mutations in Argonaute proteins (AGOs) cause Argonaute syndrome, a neurodevelopmental disorder (NDD), yet the underlying molecular mechanism remains unclear. We determined cryo-electron microscopy (cryo-EM) structures of wild-type (WT) AGO1 and the recurrent, severe causative ΔF180 mutant. AGO1(ΔF180) unexpectedly rearranges the hydrophobic core of the L1 domain to preserve the overall RNA-induced silencing complexes (RISC) architecture and recognize guide RNAs similarly to WT. Functionally, the mutant binds target RNAs like WT but more frequently retains passenger strands, implicating a defect in RISC assembly. Biochemical assays of ΔF180 and L190P mutants revealed normal duplex loading but impaired passenger ejection, exposing guide 3' ends to 3'→5' exonucleases and generating abnormally short ~11-nucleotide RNAs. In cells, AGO1(ΔF180), AGO1(G199S), and corresponding AGO2 mutants likewise produced truncated guides. Together, these findings support a model in which defective passenger ejection underlies the pathology of AGO-associated NDDs by rendering microRNAs bound to AGO syndrome mutants susceptible to aberrant 3' trimming.
External linksProc Natl Acad Sci U S A / PubMed:41237208 / PubMed Central
MethodsEM (single particle)
Resolution3.3 Å
Structure data

72192

9q3f

EMDB-72192, PDB-9q3f:
Structure of neurodevelopmental mutant AGO1 F180del in complex with guide RNA
Method: EM (single particle) / Resolution: 3.3 Å

72193

9q3g

EMDB-72193, PDB-9q3g:
Cryo-EM structure of human AGO1 in complex with guide RNA
Method: EM (single particle) / Resolution: 3.3 Å

Source
  • homo sapiens (human)
  • trichoplusia ni (cabbage looper)
KeywordsGENE REGULATION / Complex / mutant

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