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Record W4408610786 · doi:10.1371/journal.pone.0319401

Differential gene expression patterns in Niemann-Pick Type C and Tay-Sachs diseases: Implications for neurodegenerative mechanisms

2025· article· en· W4408610786 on OpenAlexaff
Ramin Yousefpour Shahrivar, Fatemeh Karami, Ebrahim Karami

Bibliographic record

VenuePLoS ONE · 2025
Typearticle
Languageen
FieldMedicine
TopicLysosomal Storage Disorders Research
Canadian institutionsMemorial University of Newfoundland
Fundersnot available
KeywordsGene expressionBiologyGeneComputational biologyGene expression profilingGeneticsRegulation of gene expressionBioinformatics

Abstract

fetched live from OpenAlex

Lysosomal storage disorders (LSDs) are a group of rare genetic conditions characterized by the impaired function of enzymes responsible for lipid digestion. Among these LSDs, Tay-Sachs disease (TSD) and Niemann-Pick type C (NPC) may share a common gene expression profile. In this study, we conducted a bioinformatics analysis to explore the gene expression profile overlap between TSD and NPC. Analyses were performed on RNA-seq datasets for both TSD and NPC from the Gene Expression Omnibus (GEO) database. Datasets were subjected to differential gene expression analysis utilizing the DESeq2 package in the R programming language. A total of 147 differentially expressed genes (DEG) were found to be shared between the TSD and NPC datasets. Enrichment analysis was then performed on the DEGs. We found that the common DEGs are predominantly associated with processes such as cell adhesion mediated by integrin, cell-substrate adhesion, and urogenital system development. Furthermore, construction of protein-protein interaction (PPI) networks using the Cytoscape software led to the identification of four hub genes: APOE, CD44, SNCA, and ITGB5. Those hub genes not only can unravel the pathogenesis of related neurologic diseases with common impaired pathways, but also may pave the way towards targeted gene therapy of LSDs.In addition, they serve as the potential biomarkers for related neurodegenerative diseases warranting further investigations.

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 machine prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.

metaresearch head score (Codex)0.000
metaresearch head score (Gemma)0.001
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.001
Threshold uncertainty score0.003

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0000.001
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0010.001
Bibliometrics0.0010.002
Science and technology studies0.0000.000
Scholarly communication0.0000.000
Open science0.0000.000
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0010.000

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 head0.048
GPT teacher head0.307
Teacher spread0.259 · 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; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designObservational
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

Citations2
Published2025
Admission routes1
Has abstractyes

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Same venuePLoS ONESame topicLysosomal Storage Disorders ResearchFrench-language works237,207