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GeneE
25 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
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RPL26
ribosomal protein L26
Chromosome 17 Β· 17p13.1
NCBI Gene: 6154Ensembl: ENSG00000161970.16HGNC: HGNC:10327UniProt: P61254
224PubMed Papers
21Diseases
6Drugs
5Pathogenic Variants
FUNCTIONAL ROLE
Highly ConstrainedHub Gene
RESEARCH IMPACT
Trending
CLINICAL
FDA Approved TargetOMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
RNA bindingstructural constituent of ribosomeprotein bindingmRNA 5'-UTR bindingBlackfan-Diamond anemiaDiamond-Blackfan anemiaminimally differentiated acute myeloblastic leukemiainfluenza
✦AI Summary

RPL26 is a ribosomal protein component of the large (60S) ribosomal subunit that plays critical roles in protein synthesis and quality control mechanisms 1. As a structural constituent of the ribosome, RPL26 participates in the basic process of protein translation 1. However, RPL26 has additional specialized functions beyond its structural role. It serves as the principal target of UFMylation, a ubiquitin-like modification that occurs predominantly on ER membrane-bound ribosomes and polysomes 1. This UFMylation is catalyzed by enzyme complexes tethered to the ER surface and appears to facilitate cotranslational protein translocation into the ER 1. RPL26 also functions in translational regulation of p53 mRNA following DNA damage, where it enhances p53 translation through interactions with both 5'- and 3'-UTRs of p53 mRNA 2. The UFMylation of RPL26 is essential for ribosome-associated quality control (ER-RQC) at the endoplasmic reticulum, where it coordinates the degradation of arrest peptides from stalled ribosomes 34. Clinically, RPL26 is associated with Diamond-Blackfan anemia 11, and its UFMylation appears important for liver homeostasis and hepatocyte differentiation 5.

Sources cited
1
RPL26 is the principal target of UFMylation and is enriched on ER membrane-bound ribosomes
PMID: 30626644
2
RPL26 enhances p53 translation after DNA damage through UTR interactions
PMID: 22433872
3
UFMylation of RPL26 is required for ribosome-associated quality control at the ER
PMID: 40315331
4
RPL26 UFMylation is involved in ER-RQC and proteostasis
PMID: 37595036
5
RPL26 UFMylation is important for liver homeostasis and hepatocyte differentiation
PMID: 40347470
Disease Associationsβ“˜21
Blackfan-Diamond anemiaOpen Targets
0.71Strong
Diamond-Blackfan anemiaOpen Targets
0.59Moderate
influenzaOpen Targets
0.46Moderate
minimally differentiated acute myeloblastic leukemiaOpen Targets
0.46Moderate
Duchenne muscular dystrophyOpen Targets
0.46Moderate
cystic fibrosisOpen Targets
0.39Weak
aniridiaOpen Targets
0.10Suggestive
Becker muscular dystrophyOpen Targets
0.10Suggestive
non-Hodgkins lymphomaOpen Targets
0.09Suggestive
genetic disorderOpen Targets
0.09Suggestive
hepatitis B virus infectionOpen Targets
0.08Suggestive
Alport syndromeOpen Targets
0.07Suggestive
arthritisOpen Targets
0.07Suggestive
cystinosisOpen Targets
0.07Suggestive
diffuse large B-cell lymphomaOpen Targets
0.07Suggestive
Dravet syndromeOpen Targets
0.07Suggestive
epilepsyOpen Targets
0.07Suggestive
hemophilia AOpen Targets
0.07Suggestive
hemophilia BOpen Targets
0.07Suggestive
muscular dystrophyOpen Targets
0.07Suggestive
Diamond-Blackfan anemia 11UniProt
Pathogenic Variants5
NM_000987.5(RPL26):c.-6+3_-6+25delLikely pathogenic
Diamond-Blackfan anemia 11
β˜…β˜†β˜†β˜†2023
NM_000987.5(RPL26):c.-5-2A>GLikely pathogenic
Diamond-Blackfan anemia 11
β˜…β˜†β˜†β˜†2023
NM_000987.5(RPL26):c.120_121del (p.Lys41fs)Pathogenic
Diamond-Blackfan anemia 11
β˜…β˜†β˜†β˜†2023β†’ Residue 41
NM_000987.5(RPL26):c.87del (p.Met30fs)Pathogenic
Diamond-Blackfan anemia 11
β˜…β˜†β˜†β˜†2023β†’ Residue 30
NM_000987.5(RPL26):c.36_39del (p.Ser12fs)Likely pathogenic
Diamond-Blackfan anemia 11
β˜…β˜†β˜†β˜†β†’ Residue 12
View on ClinVar β†—
Drug Targets6
ATALURENApproved
80S Ribosome modulator
Duchenne muscular dystrophy
CITATUZUMAB BOGATOXPhase I
Epithelial cell adhesion molecule binding agent
ELX-02Phase II
80S Ribosome modulator
cystic fibrosis
MT-3724Phase II
80S Ribosome inhibitor
diffuse large B-cell lymphoma
TELIMOMAB ARITOXPhase II
T-cell surface glycoprotein CD5 binding agent
hepatitis B virus infection
ZOLIMOMAB ARITOXPhase II
T-cell surface glycoprotein CD5 binding agent
arthritis
Related Genes
BTF3Protein interaction100%ETF1Protein interaction100%EIF6Protein interaction100%NACAProtein interaction100%PA2G4Protein interaction100%RPL17Protein interaction100%
Tissue Expression6 tissues
Ovary
100%
Brain
56%
Bone Marrow
52%
Lung
37%
Liver
34%
Heart
18%
Gene Interaction Network
Click a node to explore
RPL26BTF3ETF1EIF6NACAPA2G4RPL17
PROTEIN STRUCTURE
Preparing viewer…
PDB8A3D Β· 1.67 Γ… Β· EM
View on RCSB β†—
Constraintβ“˜
LOEUFβ“˜
0.26Highly Constrained
pLIβ“˜
0.99Intolerant
Observed/Expected LoF0.00 [0.00–0.26]
RankingsWhere RPL26 stands among ~20K protein-coding genes
  • #1,820of 20,598
    Most Researched224 Β· top 10%
  • #849of 1,025
    FDA-Approved Drug Targets1
  • #3,479of 5,498
    Most Pathogenic Variants5
  • #825of 17,882
    Most Constrained (LOEUF)0.26 Β· top 5%
Genes detectedRPL26
Sources retrieved25 papers
Response timeβ€”
πŸ“„ Sources
25β–Ό
1
A Genome-wide ER-phagy Screen Highlights Key Roles of Mitochondrial Metabolism and ER-Resident UFMylation.
PMID: 32160526
Cell Β· 2020
1.00
2
VCP/p97 UFMylation stabilizes BECN1 and facilitates the initiation of autophagy.
PMID: 38762759
Autophagy Β· 2024
0.90
3
Ribosomal protein RPL26 is the principal target of UFMylation.
PMID: 30626644
Proc Natl Acad Sci U S A Β· 2019
0.80
4
Stable Cas9 expression regulates cell growth by facilitating mTORC2 activation.
PMID: 41020501
Nucleic Acids Res Β· 2025
0.72
5
Interactions of nucleolin and ribosomal protein L26 (RPL26) in translational control of human p53 mRNA.
PMID: 22433872
J Biol Chem Β· 2012
0.70