Integrative transcriptomic profiling reveals convergent coding and non-coding signatures in pediatric bone marrow failure syndromes

Abstract Pediatric bone marrow failure (BMF) comprises heterogeneous inherited and immune-mediated disorders, yet many cases remain genetically unclassified despite comprehensive genomic evaluation. To identify shared transcriptional features beyond protein-coding variation, we performed total RNA sequencing on bone marrow samples from pediatric BMF patients (n=12) and controls (n=2). Based on whole-genome sequencing, patients were stratified into genetically defined BMF (g-BMF, n=4) and genetically undefined BMF (u-BMF, n=8). Comparative transcriptomic analyses revealed minimal differences in protein-coding genes and long non-coding RNAs (lncRNAs) between g-BMF and u-BMF, indicating strong transcriptional concordance. Compared with controls, 55.2% of differentially expressed protein-coding genes were shared between g-BMF and u-BMF and were predominantly upregulated. Pathway enrichment analyses consistently identified immune activation and cellular stress-response pathways, suggesting a shared inflammatory transcriptional state across pediatric BMF irrespective of genetic classification. LncRNA profiling showed that 69.6% of differentially expressed lncRNAs were common to both BMF groups. ATP1A1-AS1, USP3-AS1 , and SNHG32 were reproducibly overexpressed and validated by RT–qPCR. Correlation-based pathway analyses associated ATP1A1-AS1 and USP3-AS1 with immune-related and biosynthetic programs, whereas SNHG32 showed a distinct co-expression pattern. Collectively, these findings indicate shared coding and non-coding transcriptional features in pediatric BMF that appear largely independent of genetic classification.

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Journal
Scientific Reports
Published
2026-08-27
DOI
https://doi.org/10.1038/s41598-026-68248-5
Primary Topic
Autoimmune and Inflammatory Disorders Research
Type
article
Field-Weighted Citation Impact
0.00

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article

Integrative transcriptomic profiling reveals convergent coding and non-coding signatures in pediatric bone marrow failure syndromes

Hyunjoo Bae, Jae Won Yoo, Jaeeun Yoo, Jong‐Mi Lee et al.
Scientific Reports
Autoimmune and Inflammatory Disorders Research
article

Integrative transcriptomic profiling reveals convergent coding and non-coding signatures in pediatric bone marrow failure syndromes

Hyunjoo Bae, Jae Won Yoo, Jaeeun Yoo, Jong‐Mi Lee, Myungshin Kim, Jae Wook Lee, Nack-Gyun Chung, Jae Wook Lee, Myungshin Kim
article en

Abstract

Abstract Pediatric bone marrow failure (BMF) comprises heterogeneous inherited and immune-mediated disorders, yet many cases remain genetically unclassified despite comprehensive genomic evaluation. To identify shared transcriptional features beyond protein-coding variation, we performed total RNA sequencing on bone marrow samples from pediatric BMF patients (n=12) and controls (n=2). Based on whole-genome sequencing, patients were stratified into genetically defined BMF (g-BMF, n=4) and genetically undefined BMF (u-BMF, n=8). Comparative transcriptomic analyses revealed minimal differences in protein-coding genes and long non-coding RNAs (lncRNAs) between g-BMF and u-BMF, indicating strong transcriptional concordance. Compared with controls, 55.2% of differentially expressed protein-coding genes were shared between g-BMF and u-BMF and were predominantly upregulated. Pathway enrichment analyses consistently identified immune activation and cellular stress-response pathways, suggesting a shared inflammatory transcriptional state across pediatric BMF irrespective of genetic classification. LncRNA profiling showed that 69.6% of differentially expressed lncRNAs were common to both BMF groups. ATP1A1-AS1, USP3-AS1 , and SNHG32 were reproducibly overexpressed and validated by RT–qPCR. Correlation-based pathway analyses associated ATP1A1-AS1 and USP3-AS1 with immune-related and biosynthetic programs, whereas SNHG32 showed a distinct co-expression pattern. Collectively, these findings indicate shared coding and non-coding transcriptional features in pediatric BMF that appear largely independent of genetic classification.

Scientific Reports
The Catholic University of Korea Incheon St. Mary's Hospital (KR), The Catholic University of Korea Seoul St. Mary's Hospital (KR), Catholic University of Korea (KR)
Austrian Science Fund, Fonds Wetenschappelijk Onderzoek, National Research Foundation of Korea, Vlaamse regering
Good health and well-being
Openalex Percentile: Top 67%
Autoimmune and Inflammatory Disorders Research
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