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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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RBM26
RNA binding motif protein 26
Chromosome 13 · 13q31.1
NCBI Gene: 64062Ensembl: ENSG00000139746.16HGNC: HGNC:20327UniProt: A0A087X0H9
101PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingRNA bindingnucleusnucleoplasmneurodegenerative diseaseschizophreniasubstance-related disorderadolescent idiopathic scoliosis
✦AI Summary

RBM26 (RNA binding motif protein 26) is a nuclear RNA-binding protein that plays critical roles in RNA metabolism and quality control. The protein functions as an essential component of the Poly(A) Tail eXosome Targeting (PAXT) complex, which facilitates the degradation of nuclear polyadenylated RNA by recruiting the RNA exosome 1. RBM26, along with RBM27, can be recruited by PABPN1 to promote splicing of terminal introns, particularly those with weak 3' splice sites 2. The protein also participates in nuclear RNA sorting mechanisms, where it acts as a transient PAXT component that helps trigger exosomal degradation of shorter RNAs while allowing longer RNAs to proceed to nuclear export 3. Disease relevance studies have identified RBM26 as a significant risk gene in genome-wide association studies for tic disorders, suggesting its importance in neurological function 4. Additionally, RBM26 variants have been associated with coal workers' pneumoconiosis risk in Chinese populations, with the gene showing lung tissue-specific expression patterns 5. The protein's RNA-binding and regulatory functions position it as a key player in nuclear RNA surveillance and processing pathways.

Sources cited
1
RBM26 is a critical component of the PAXT complex that facilitates nuclear RNA degradation by the exosome
PMID: 31950173
2
RBM26 can be recruited by PABPN1 to promote splicing of terminal introns with weak 3' splice sites
PMID: 37661812
3
RBM26 acts as a transient PAXT component in nuclear RNA sorting, helping trigger degradation of shorter RNAs
PMID: 39461342
4
RBM26 was identified as a significant risk gene in genome-wide association studies for tic disorders
PMID: 39389409
5
RBM26 variants are associated with coal workers' pneumoconiosis risk and show lung tissue-specific expression
PMID: 29394417
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.49Moderate
schizophreniaOpen Targets
0.34Weak
substance-related disorderOpen Targets
0.31Weak
adolescent idiopathic scoliosisOpen Targets
0.30Weak
rheumatoid arthritisOpen Targets
0.18Weak
Abnormal nasolacrimal system morphologyOpen Targets
0.15Weak
Alzheimer diseaseOpen Targets
0.12Weak
chronic laryngitisOpen Targets
0.12Weak
hypertrophic cardiomyopathyOpen Targets
0.11Weak
hypertensionOpen Targets
0.10Weak
complicationOpen Targets
0.09Suggestive
hemorrhageOpen Targets
0.09Suggestive
sialolithiasisOpen Targets
0.09Suggestive
metabolic syndromeOpen Targets
0.07Suggestive
type 2 diabetes mellitusOpen Targets
0.06Suggestive
smoking behaviorOpen Targets
0.05Suggestive
skin diseaseOpen Targets
0.05Suggestive
attention deficit hyperactivity disorderOpen Targets
0.05Suggestive
substance abuseOpen Targets
0.05Suggestive
diverticular diseaseOpen Targets
0.05Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PPP1R8Shared pathway100%STK31Shared pathway100%RBM27Shared pathway75%ZC3H3Shared pathway68%ZFC3H1Protein interaction65%RNASEH2BShared pathway50%
Tissue Expression6 tissues
Bone Marrow
100%
Ovary
65%
Brain
64%
Lung
49%
Liver
49%
Heart
47%
Gene Interaction Network
Click a node to explore
RBM26PPP1R8STK31RBM27ZC3H3ZFC3H1RNASEH2B
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q5T8P6
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.42Moderately Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.28 [0.19–0.42]
RankingsWhere RBM26 stands among ~20K protein-coding genes
  • #4,742of 20,598
    Most Researched101 · top quartile
  • #2,199of 17,882
    Most Constrained (LOEUF)0.42 · top quartile
Genes detectedRBM26
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
The polyA tail facilitates splicing of last introns with weak 3' splice sites via PABPN1.
PMID: 37661812
EMBO Rep · 2023
1.00
2
The human ZC3H3 and RBM26/27 proteins are critical for PAXT-mediated nuclear RNA decay.
PMID: 31950173
Nucleic Acids Res · 2020
0.90
3
A putative lncRNA RBM26-AS1-encoded micropeptide promotes colon cancer progression.
PMID: 40652529
Int J Surg · 2025
0.80
4
Dual modes of ZFC3H1 confer selectivity in nuclear RNA sorting.
PMID: 39461342
Mol Cell · 2024
0.70
5
Genome-Wide Association Study Meta-Analysis of 9619 Cases With Tic Disorders.
PMID: 39389409
Biol Psychiatry · 2025
0.60