ArticleGenes & development2005
Kissing complex RNAs mediate interaction between the Fragile-X mental retardation protein KH2 domain and brain polyribosomes.
Article in Genes & development, 2005. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 152 papers.
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Who cites it
152 citing papers in PubMed, 289 citations in OpenAlex.
- FMR1 KH0-KH1 domains coordinate mExperimental cell research · 2025Article
- Review
- FMRP cooperates with miRISC components to repress translation and regulate neurite morphogenesis inRNA biology · 2024Article
- The RNA-Binding Function of Ribosomal Proteins and Ribosome Biogenesis Factors in Human Health and Disease.Biomedicines · 2023Review
- FMRP phosphorylation and interactions with Cdh1 regulate association with dendritic RNA granules and MEF2-triggered synapse elimination.Neurobiology of disease · 2023Article
- FMRP binds Per1 mRNA and downregulates its protein expression in mice.Molecular brain · 2023Article
- The RGG motif proteins: Interactions, functions, and regulations.Wiley interdisciplinary reviews. RNA · 2023Review
- Function of FMRP Domains in Regulating Distinct Roles of Neuronal Protein Synthesis.Molecular neurobiology · 2022Article
- AGO-RBP crosstalk on target mRNAs: Implications in miRNA-guided gene silencing and cancer.Translational oncology · 2022Review
- The Fragile X Protein Disordered Regions Bind a Novel RNA Target.Biochemistry · 2022Article
- FXS causing missense mutations disrupt FMRP granule formation, dynamics, and function.PLoS genetics · 2022Article
- The Fragile X Proteins Differentially Regulate Translation of Reporter mRNAs with G-quadruplex Structures.Journal of molecular biology · 2022Article
- FMRP regulates mRNAs encoding distinct functions in the cell body and dendrites of CA1 pyramidal neurons.eLife · 2021Article
- RNA-binding proteins in neurological development and disease.RNA biology · 2021Review
- Brain Long Noncoding RNAs: Multitask Regulators of Neuronal Differentiation and Function.Molecules (Basel, Switzerland) · 2021Review
- FMRP Interacts with RARα in Synaptic Retinoic Acid Signaling and Homeostatic Synaptic Plasticity.International journal of molecular sciences · 2021Article
- An "Omic" Overview of Fragile X Syndrome.Biology · 2021Review
- The molecular biology of FMRP: new insights into fragile X syndrome.Nature reviews. Neuroscience · 2021Review
- RNA-binding proteins in human genetic disease.Nature reviews. Genetics · 2021Review
- The Human Fragile X Mental Retardation Protein Inhibits the Elongation Step of Translation through Its RGG and C-Terminal Domains.Biochemistry · 2020Article
92 more citing papers are in PubMed but not listed here.
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Authors and funding
7 authors at 3 institutions in 1 country.
Funding
Abstract
Fragile-X mental retardation is caused by loss of function of a single gene encoding the Fragile-X mental retardation protein, FMRP, an RNA-binding protein that harbors two KH-type and one RGG-type RNA-binding domains. Previous studies identified intramolecular G-quartet RNAs as high-affinity targets for the RGG box, but the relationship of RNA binding to FMRP function and mental retardation remains unclear. One severely affected patient harbors a missense mutation (I304N) within the second KH domain (KH2), and some evidence suggests this domain may be involved in the proposed role of FMRP in translational regulation. We now identify the RNA target for the KH2 domain as a sequence-specific element within a complex tertiary structure termed the FMRP kissing complex. We demonstrate that the association of FMRP with brain polyribosomes is abrogated by competition with the FMRP kissing complex RNA, but not by high-affinity G-quartet RNAs. We conclude that mental retardation associated with the I304N mutation, and likely the Fragile-X syndrome more generally, may relate to a crucial role for RNAs harboring the kissing complex motif as targets for FMRP translational regulation.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.