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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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DGCR8
DGCR8 microprocessor complex subunit
Chromosome 22 · 22q11.21
NCBI Gene: 54487Ensembl: ENSG00000128191.18HGNC: HGNC:2847UniProt: Q8WYQ5
151PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Highly Constrained
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
primary miRNA processingDNA damage responsepositive regulation of pre-miRNA processingnucleusneurodegenerative diseaseNephroblastomasmoking initiationPleural Mesothelioma
✦AI Summary

DGCR8 is a critical component of the microprocessor complex that plays an essential role in microRNA (miRNA) biogenesis. The protein functions as part of a complex with DROSHA to process primary miRNA transcripts (pri-miRNAs) into precursor miRNAs (pre-miRNAs) in the nucleus 1. DGCR8 acts as a molecular anchor that recognizes pri-miRNAs at dsRNA-ssRNA junctions and directs DROSHA to cleave appropriately for pre-miRNA release. The protein exhibits heme-binding properties, with heme-bound DGCR8 dimers forming cooperative trimers that are highly active in pri-miRNA processing 2. DGCR8 specifically recognizes N6-methyladenosine (m6A)-modified pri-miRNAs, which is required for efficient processing, and interacts with HNRNPA2B1 to mediate m6A-dependent nuclear RNA processing events 2. The protein also participates in global miRNA dosage control through a developmentally regulated mechanism involving alternative transcription initiation, which maintains proper DGCR8:DROSHA stoichiometry and prevents microprocessor aggregation 3. Dysregulation of this dosage control affects lipid metabolic pathways and disrupts embryonic germ layer specification. DGCR8 mutations, particularly the E518K variant, are associated with increased thyroid cancer risk 4. Knockout studies demonstrate DGCR8's essential role in canonical miRNA production and cellular differentiation processes 5.

Sources cited
1
DGCR8 functions as part of the microprocessor complex with DROSHA to process pri-miRNAs into pre-miRNAs
PMID: 26662984
2
DGCR8 interacts with HNRNPA2B1 and recognizes m6A-modified pri-miRNAs for processing
PMID: 26321680
3
DGCR8 participates in global miRNA dosage control through alternative transcription initiation
PMID: 33953397
4
DGCR8 mutations, particularly E518K, are associated with increased thyroid cancer risk
PMID: 38607000
5
DGCR8 knockout demonstrates its essential role in canonical miRNA production
PMID: 30694346
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.54Moderate
NephroblastomaOpen Targets
0.37Weak
smoking initiationOpen Targets
0.31Weak
Pleural MesotheliomaOpen Targets
0.27Weak
diffuse large B-cell lymphomaOpen Targets
0.23Weak
neoplasmOpen Targets
0.20Weak
hepatocellular carcinomaOpen Targets
0.20Weak
head and neck squamous cell carcinomaOpen Targets
0.20Weak
acute myeloid leukemiaOpen Targets
0.19Weak
gliomaOpen Targets
0.19Weak
esophageal squamous cell carcinomaOpen Targets
0.19Weak
melanomaOpen Targets
0.19Weak
clear cell renal carcinomaOpen Targets
0.19Weak
urinary bladder carcinomaOpen Targets
0.19Weak
skin basal cell carcinomaOpen Targets
0.19Weak
lung adenocarcinomaOpen Targets
0.19Weak
breast carcinomaOpen Targets
0.19Weak
colorectal adenocarcinomaOpen Targets
0.19Weak
chronic lymphocytic leukemiaOpen Targets
0.19Weak
thyroid carcinomaOpen Targets
0.19Weak
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
HNRNPDLProtein interaction99%DDX17Protein interaction99%XPO5Protein interaction99%DDX5Protein interaction98%FMR1Protein interaction98%KHSRPProtein interaction98%
Tissue Expression6 tissues
Ovary
100%
Bone Marrow
92%
Liver
90%
Lung
64%
Heart
53%
Brain
42%
Gene Interaction Network
Click a node to explore
DGCR8HNRNPDLDDX17XPO5DDX5FMR1KHSRP
PROTEIN STRUCTURE
Preparing viewer…
PDB7CNC · 1.60 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.27Highly Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.17 [0.11–0.27]
RankingsWhere DGCR8 stands among ~20K protein-coding genes
  • #2,993of 20,598
    Most Researched151 · top quartile
  • #908of 17,882
    Most Constrained (LOEUF)0.27 · top 10%
Genes detectedDGCR8
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
SIRT6-regulated macrophage efferocytosis epigenetically controls inflammation resolution of diabetic periodontitis.
PMID: 36593966
Theranostics · 2023
1.00
2
HNRNPA2B1 Is a Mediator of m(6)A-Dependent Nuclear RNA Processing Events.
PMID: 26321680
Cell · 2015
0.90
3
DICER1 and DGCR8 in thyroid tumorigenesis: miRNA biogenesis and histopathologic diversity.
PMID: 41104964
Eur Thyroid J · 2025
0.80
4
Unraveling the Significance of DGCR8 and miRNAs in Thyroid Carcinoma.
PMID: 38607000
Cells · 2024
0.70
5
A functional connection between the Microprocessor and a variant NEXT complex.
PMID: 39515294
Mol Cell · 2024
0.60