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5 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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ZBTB8OS
zinc finger and BTB domain containing 8 opposite strand
Chromosome 1 · 1p35.1
NCBI Gene: 339487Ensembl: ENSG00000176261.16HGNC: HGNC:24094UniProt: A0A8C8MQ05
25PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein guanylyltransferase activityRNA ligase (GTP) activityprotein bindingRNA splicing, via endonucleolytic cleavage and ligationhemolytic anemiaAbnormality of the skeletal systemtype 2 diabetes mellitusacute lymphoblastic leukemia
✦AI Summary

ZBTB8OS (also known as archease) is a cofactor essential for activating RTCB, the catalytic subunit of the tRNA-splicing ligase complex involved in pre-tRNA maturation 1. ZBTB8OS catalyzes GTP-dependent activation of RTCB, facilitating the formation of an RTCB-guanylate intermediate required for RNA ligation 1. The protein functions in two distinct RNA splicing pathways: canonical tRNA splicing and non-canonical XBP1 mRNA splicing. In tRNA maturation, ZBTB8OS enables ligation of exon halves generated after intron removal by the tRNA-splicing endonuclease 1. During endoplasmic reticulum (ER) stress, ZBTB8OS is required for XBP1 mRNA splicing via the unfolded protein response (UPR), where it cooperates with RTCB to ligate exon ends after IRE1/ERN1-mediated intron removal 2. This XBP1 splicing generates the active XBP1s transcription factor, which induces genes resolving protein folding defects 2. Structurally, ZBTB8OS contains an extended N-terminal domain and a compacted C-terminal domain that interact with and stabilize RTCB in cells 3. Loss of ZBTB8OS function impairs antibody secretion in plasma cells and represents a potential therapeutic target for diseases involving elevated XBP1s expression 2.

Sources cited
1
ZBTB8OS (archease) is required for full activity of tRNA ligase complex and facilitates RTCB-guanylate intermediate formation
PMID: 24870230
2
ZBTB8OS has N-terminal and C-terminal domains that interact with RTCB and are important for protein stability
PMID: 32234548
3
ZBTB8OS (archease) with RTCB mediates XBP1 mRNA splicing during UPR and depletion impairs antibody secretion in plasma cells
PMID: 25378478
⚠Limited data available — This gene has 3 indexed publications. Summary and analysis may be incomplete.
Disease Associationsⓘ20
hemolytic anemiaOpen Targets
0.27Weak
Abnormality of the skeletal systemOpen Targets
0.06Suggestive
type 2 diabetes mellitusOpen Targets
0.02Suggestive
acute lymphoblastic leukemiaOpen Targets
0.02Suggestive
acute myeloid leukemiaOpen Targets
0.02Suggestive
AnxietyOpen Targets
0.01Suggestive
Chédiak-Higashi syndromeOpen Targets
0.01Suggestive
chronic myelomonocytic leukemiaOpen Targets
0.01Suggestive
hematopoietic and lymphoid cell neoplasmOpen Targets
0.01Suggestive
cancerOpen Targets
0.01Suggestive
cardiovascular diseaseOpen Targets
0.01Suggestive
myelodysplastic syndromeOpen Targets
0.00Suggestive
congestive heart failureOpen Targets
0.00Suggestive
syphilisOpen Targets
0.00Suggestive
myeloid neoplasmOpen Targets
0.00Suggestive
viral diseaseOpen Targets
0.00Suggestive
atherosclerosisOpen Targets
0.00Suggestive
hematologic diseaseOpen Targets
0.00Suggestive
primary myelofibrosisOpen Targets
0.00Suggestive
strokeOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
DDX1Protein interaction100%RTCBProtein interaction81%FAM98BProtein interaction75%C2orf49Protein interaction72%RTRAFProtein interaction70%TSEN2Shared pathway50%
Tissue Expression6 tissues
Liver
100%
Brain
91%
Lung
85%
Heart
74%
Bone Marrow
69%
Ovary
59%
Gene Interaction Network
Click a node to explore
ZBTB8OSDDX1RTCBFAM98BC2orf49RTRAFTSEN2
PROTEIN STRUCTURE
Preparing viewer…
PDB8BTX · 1.84 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.16LoF Tolerant
pLIⓘ
0.02Tolerant
Observed/Expected LoF0.62 [0.35–1.16]
RankingsWhere ZBTB8OS stands among ~20K protein-coding genes
  • #13,121of 20,598
    Most Researched25
  • #12,123of 17,882
    Most Constrained (LOEUF)1.16
Genes detectedZBTB8OS
Sources retrieved5 papers
Response time—
📄 Sources
5
1
Analysis of orthologous groups reveals archease and DDX1 as tRNA splicing factors.
PMID: 24870230
Nature · 2014
1.00
2
Age-related mutations and chronic myelomonocytic leukemia.
PMID: 26648538
Leukemia · 2016
0.80
3
Crystal structure of human archease, a key cofactor of tRNA splicing ligase complex.
PMID: 32234548
Int J Biochem Cell Biol · 2020
0.60
4
Making ends meet: a role of RNA ligase RTCB in unfolded protein response.
PMID: 25404664
EMBO J · 2014
0.40
5
The mammalian tRNA ligase complex mediates splicing of XBP1 mRNA and controls antibody secretion in plasma cells.
PMID: 25378478
EMBO J · 2014
0.20