ArticleThe Journal of general physiology2020
Differential regulation of BK channels by fragile X mental retardation protein.
Article in The Journal of general physiology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 22 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
22 citing papers in PubMed, 29 citations in OpenAlex.
- SPG601-associated modulation of resting-state EEG and improvement in executive function in a fragile X syndrome randomized controlled crossover study.Scientific reports · 2026Trial
- Associations Between Altered Auditory EEG Markers and Clinical Impairments in Fragile X Syndrome.Journal of autism and developmental disorders · 2025Article
- GRIN2B disease-associated mutations disrupt the function of BK channels and NMDA receptor signaling nanodomains.The Journal of general physiology · 2025Article
- From Discovery to Innovative Translational Approaches in 80 Years of Fragile X Syndrome Research.Biomedicines · 2025Review
- Therapeutic efficacy of the BKCa channel opener chlorzoxazone in a mouse model of Fragile X syndrome.Neuropsychopharmacology : official publication of the American College of Neuropsychopharmacology · 2024Article
- Subunit-specific inhibition of BK channels by piperine.Biophysical journal · 2024Article
- Altered brain serotonin 5-HTNeuropharmacology · 2024Article
- Large conductance voltage-and calcium-activated KFrontiers in pharmacology · 2024Review
- Hyperacusis in the AdultInternational journal of molecular sciences · 2023Article
- Characterization of Fragile X Mental Retardation Protein expression in human nociceptors and their axonal projections to the spinal dorsal horn.The Journal of comparative neurology · 2023Article
- CaInternational journal of molecular sciences · 2023Review
- Review
- From circuits to behavior: Amygdala dysfunction in fragile X syndrome.Frontiers in integrative neuroscience · 2023Review
- Neurodevelopment and early pharmacological interventions in Fragile X Syndrome.Frontiers in neuroscience · 2023Review
- Article
- The role of altered translation in intellectual disability and epilepsy.Progress in neurobiology · 2022Review
- FMRP Sustains Presynaptic Function via Control of Activity-Dependent Bulk Endocytosis.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2022Article
- A Double Jeopardy: Loss of FMRP Results in DSB and Down-regulated DNA Repair.21st century pathology · 2022Article
- Effect of an autism-associatedCurrent research in physiology · 2022Article
- An emerging spectrum of variants and clinical features inChannels (Austin, Tex.) · 2021Review
Corrections and comments
- Commented on by
- Erratum issued
Authors and funding
6 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Fragile X mental retardation protein (FMRP) is an RNA-binding protein prominently expressed in neurons. Missense mutations or complete loss of FMRP can potentially lead to fragile X syndrome, a common form of inherited intellectual disability. In addition to RNA regulation, FMRP was also proposed to modulate neuronal function by direct interaction with the large conductance Ca2+- and voltage-activated potassium channel (BK) β4 regulatory subunits (BKβ4). However, the molecular mechanisms underlying FMRP regulation of BK channels were not studied in detail. We have used electrophysiology and super-resolution stochastic optical reconstruction microscopy (STORM) to characterize the effects of FMRP on pore-forming BKα subunits, as well as the association with regulatory subunits BKβ4. Our data indicate that, in the absence of coexpressed β4, FMRP alters the steady-state properties of BKα channels by decreasing channel activation and deactivation rates. Analysis using the Horrigan-Aldrich model revealed alterations in the parameters associated with channel opening (L0) and voltage sensor activation (J0). Interestingly, FMRP also altered the biophysical properties of BKαβ4 channels favoring channel opening, although not as dramatically as BKα. STORM experiments revealed clustered multi-protein complexes, consistent with FMRP interacting not only to BKαβ4 but also to BKα. Lastly, we found that a partial loss-of-function mutation in FMRP (R138Q) counteracts many of its functional effects on BKα and BKαβ4 channels. In summary, our data show that FMRP modulates the function of both BKα and BKαβ4 channels.
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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.