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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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RBM7
RNA binding motif protein 7
Chromosome 11 · 11q23.2
NCBI Gene: 10179Ensembl: ENSG00000076053.12HGNC: HGNC:9904UniProt: G3V1T9
90PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
nucleolus14-3-3 protein bindingpre-mRNA intronic bindingsnRNA catabolic processneurodegenerative diseasebreast cancerSenior-Boichis syndromehepatorenocardiac degenerative fibrosis
✦AI Summary

RBM7 (RNA binding motif protein 7) is an RNA-binding subunit of the trimeric nuclear exosome targeting (NEXT) complex that directs specific non-coding RNAs for degradation by the RNA exosome 1. The protein functions as a critical component in RNA surveillance, binding preferentially to polyuridine sequences and associating with newly synthesized RNAs including promoter upstream transcripts (PROMPTs), enhancer RNAs (eRNAs), and 3'-extended snRNA products 2. Structurally, RBM7 contains an RRM domain and works within NEXT alongside MTR4 helicase and ZCCHC8, with all three subunits collaborating in RNA binding and substrate recognition 2. Interestingly, RBM7 can be replaced by DGCR8/DROSHA in a variant NEXT complex that specifically targets stem-loop-containing RNAs from enhancers and other genomic regions 3. Beyond RNA surveillance, RBM7 plays important roles in cancer biology, where its deficiency promotes breast cancer metastasis through coordinated MFGE8 splicing switches and NF-κB pathway activation 4. In glioblastoma, RBM7 enhances temozolomide resistance by suppressing mitochondrial dysfunction and ferroptosis through destabilization of FBXL16 mRNA 5. Additionally, RBM7 contributes to lung fibrosis pathogenesis by promoting epithelial cell apoptosis through NEAT1 decay 6.

Sources cited
1
RBM7 is a component of the nuclear exosome targeting (NEXT) complex involved in RNA degradation
PMID: 27139832
2
RBM7 collaborates with MTR4 and ZCCHC8 in RNA binding and substrate recognition within NEXT complex
PMID: 35688134
3
RBM7 can be replaced by DGCR8/DROSHA in variant NEXT complex targeting stem-loop RNAs
PMID: 39515294
4
RBM7 deficiency promotes breast cancer metastasis through MFGE8 splicing control and NF-κB pathway
PMID: 38995840
5
RBM7 enhances glioblastoma temozolomide resistance by destabilizing FBXL16 mRNA and suppressing ferroptosis
PMID: 41381783
6
RBM7 promotes lung fibrosis by causing NEAT1 decay and epithelial cell apoptosis
PMID: 34165514
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.42Moderate
breast cancerOpen Targets
0.10Suggestive
Senior-Boichis syndromeOpen Targets
0.05Suggestive
hepatorenocardiac degenerative fibrosisOpen Targets
0.04Suggestive
neoplasmOpen Targets
0.04Suggestive
pulmonary fibrosis and/or bone marrow failure syndrome, telomere-related, 9Open Targets
0.04Suggestive
nephronophthisis 3Open Targets
0.04Suggestive
polycystic kidney disease 4Open Targets
0.03Suggestive
immunodeficiency 73b with defective neutrophil chemotaxis and lymphopeniaOpen Targets
0.03Suggestive
triple-negative breast cancerOpen Targets
0.03Suggestive
idiopathic CD4 lymphocytopeniaOpen Targets
0.03Suggestive
pulmonary fibrosis and/or bone marrow failure, Telomere-related, 3Open Targets
0.03Suggestive
familial juvenile hyperuricemic nephropathy type 2Open Targets
0.03Suggestive
Hyperuricemia - anemia - renal failureOpen Targets
0.03Suggestive
Dent diseaseOpen Targets
0.03Suggestive
Dent disease type 1Open Targets
0.03Suggestive
idiopathic pulmonary fibrosisOpen Targets
0.03Suggestive
interstitial lung disease 1Open Targets
0.03Suggestive
sclerosing cholangitisOpen Targets
0.03Suggestive
neuroendocrine neoplasmOpen Targets
0.02Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
EXOSC1Protein interaction100%MPHOSPH6Protein interaction100%C1DProtein interaction100%EXOSC10Protein interaction100%EXOSC3Protein interaction100%EXOSC2Protein interaction100%
Tissue Expression6 tissues
Bone Marrow
100%
Liver
50%
Ovary
44%
Lung
42%
Brain
38%
Heart
36%
Gene Interaction Network
Click a node to explore
RBM7EXOSC1MPHOSPH6C1DEXOSC10EXOSC3EXOSC2
PROTEIN STRUCTURE
Preparing viewer…
PDB5LXR · 2.00 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.77LoF Tolerant
pLIⓘ
0.09Tolerant
Observed/Expected LoF0.46 [0.29–0.77]
RankingsWhere RBM7 stands among ~20K protein-coding genes
  • #5,333of 20,598
    Most Researched90
  • #6,165of 17,882
    Most Constrained (LOEUF)0.77
Genes detectedRBM7
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
A 20-year Clinical and Genetic Neuromuscular Cohort Analysis in Lebanon: An International Effort.
PMID: 34602496
J Neuromuscul Dis · 2022
1.00
2
RRM domain of human RBM7: purification, crystallization and structure determination.
PMID: 27139832
Acta Crystallogr F Struct Biol Commun · 2016
0.90
3
Structural basis for RNA surveillance by the human nuclear exosome targeting (NEXT) complex.
PMID: 35688134
Cell · 2022
0.80
4
A functional connection between the Microprocessor and a variant NEXT complex.
PMID: 39515294
Mol Cell · 2024
0.70
5
RBM7 deficiency promotes breast cancer metastasis by coordinating MFGE8 splicing switch and NF-kB pathway.
PMID: 38995840
Elife · 2024
0.60