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Record W7112701537

Testing the interaction of HEPN domain of Sacsin with RNA through experimental and computational efforts

2025· book-chapter· en· W7112701537 on OpenAlexaboutno aff

Bibliographic record

VenueNova Science Publishers (Nova Science Publishers, Inc.) · 2025
Typebook-chapter
Languageen
FieldNeuroscience
TopicGenetic Neurodegenerative Diseases
Canadian institutionsnot available
Fundersnot available
KeywordsRNAMissense mutationDimerPoint mutationGeneMutationDomain (mathematical analysis)Pipeline (software)
DOInot available

Abstract

fetched live from OpenAlex

Autosomal recessive spastic ataxia of Charlevoix-Saguenay, or ARSACS, is a neurodegenerative disorder whose symptoms appear in childhood and is caused by point mutations in the SACS gene (13q11), which encodes the Sacsin protein. Sacsin includes a HEPN domain, which shares structural similarity with the HEPN domains of other known RNA-binding proteins. Notably, individuals with a single missense mutation in this domain exhibit symptoms comparable to those of other ARSACS patients, indicating that altered RNA-binding abilities may play a role in the disease’s pathogenesis. We are investigating the RNA-binding properties of the HEPN domain. With the help of bioinformatic tools RNAA and RNAB, were selected based on their predicted higher (A) and lower (B) affinity for the HEPN domain, respectively. Their interaction with HEPN was analyzed via intrinsic fluorescence, circular dichroism (CD), and size-exclusion chromatography (SEC). Here we present the results of these experiments as well as GST-pulldown assays that confirmed the ability of HEPN to selectively bind to RNAs. We also show the modelling efforts to investigate the interaction of HEPN (monomer and dimer) with short RNA sequences. We used Alphafold3 and Nufold to predict the structure of isolated RNA sequences and of HEPN-RNA complexes (Alphafold3 only). Notwithstanding the difficulty in predicting RNA 3D structures with currently available computational tools, we found a suggestion of a specific interaction at the monomer-monomer interface of the HEPN dimer. We also performed Molecular Dynamics simulations of the HEPN, HEPN dimer and of complexes with strongly and weakly interacting RNA sequences. We plan to design a pipeline of machine learning and traditional tools to explore a wider space of RNA sequences to retrieve stronger and more specific RNA binders of HEPN.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame distilled prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.

metaresearch head score (Codex)0.003
metaresearch head score (Gemma)0.004
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesMeta-epidemiology (narrow), Science and technology studies, Scholarly communication
Consensus categoriesScholarly communication
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Bench or experimental · Consensus signal: Bench or experimental
GenreCandidate signal: Empirical · Consensus signal: none
Teacher disagreement score0.503
Threshold uncertainty score1.000

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0030.004
Meta-epidemiology (narrow)0.0010.001
Meta-epidemiology (broad)0.0010.000
Bibliometrics0.0020.005
Science and technology studies0.0010.021
Scholarly communication0.0050.016
Open science0.0040.002
Research integrity0.0000.001
Insufficient payload (model declined to judge)0.0000.000

Machine scores (provisional)

The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.

Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.

Opus teacher head0.061
GPT teacher head0.304
Teacher spread0.243 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; both teacher heads agree on what is shown here.

Study designBench or experimental
Domainnot available
GenreEmpirical

How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".

Quick stats

Citations0
Published2025
Admission routes1
Has abstractyes

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