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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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PRRG4
proline rich and Gla domain 4
Chromosome 11 · 11p13
NCBI Gene: 79056Ensembl: ENSG00000135378.5HGNC: HGNC:30799UniProt: A0A0S2Z5M6
18PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
plasma membraneprotein bindingWW domain bindingGO:0005615placenta praeviarespiratory tract infectious disorderbreast cancerpapillary thyroid carcinoma
✦AI Summary

PRRG4 (proline rich and Gla domain 4) is a transmembrane protein that functions primarily as a regulator of axon guidance and cell migration through its control of the ROBO1 receptor. PRRG4 acts as a functional homolog of the Drosophila commissureless protein, preventing ROBO1 delivery to the cell surface and promoting its degradation 1. The protein contains characteristic PY motifs and WW binding domains that enable protein-protein interactions 2. Mechanistically, PRRG4 recruits the E3 ubiquitin ligase NEDD4 through its LPSY and PPPY motifs, leading to ubiquitination and degradation of ROBO1, which subsequently activates Src and FAK signaling pathways 3. PRRG4 has significant disease relevance, particularly in cancer and neurodevelopmental disorders. In breast cancer, elevated PRRG4 expression promotes metastasis through ROBO1 downregulation 3. The gene is located within the WAGR syndrome critical region, and haploinsufficiency of PRRG4 may contribute to the severe developmental delays and autistic behaviors characteristic of this syndrome 4. Clinically, PRRG4 shows promise as a potential biomarker and therapeutic target for metastatic breast cancer 3 and may serve as a prognostic marker in esophageal adenocarcinoma 5.

Sources cited
1
PRRG4 functions as a homolog of commissureless, preventing ROBO1 surface delivery and controlling axon guidance
PMID: 28859078
2
PRRG4 promotes breast cancer metastasis by recruiting NEDD4 to degrade ROBO1 and activate Src/FAK signaling
PMID: 33037408
3
PRRG4 contains WW binding motifs that enable protein-protein interactions
PMID: 23873930
4
PRRG4 haploinsufficiency contributes to developmental delays and autistic behaviors in WAGR syndrome
PMID: 24357251
5
PRRG4 serves as a prognostic marker in esophageal adenocarcinoma
PMID: 41162922
Disease Associationsⓘ20
placenta praeviaOpen Targets
0.28Weak
respiratory tract infectious disorderOpen Targets
0.28Weak
breast cancerOpen Targets
0.08Suggestive
papillary thyroid carcinomaOpen Targets
0.06Suggestive
Inguinal herniaOpen Targets
0.04Suggestive
major depressive disorderOpen Targets
0.04Suggestive
bronchial diseaseOpen Targets
0.04Suggestive
hemoglobin H diseaseOpen Targets
0.03Suggestive
glycoprotein storage diseaseOpen Targets
0.03Suggestive
dominant beta-thalassemiaOpen Targets
0.03Suggestive
Hemoglobin C - beta-thalassemiaOpen Targets
0.03Suggestive
hemoglobin C-beta-thalassemia syndromeOpen Targets
0.03Suggestive
congenital dyserythropoietic anemia type 2Open Targets
0.03Suggestive
Congenital dyserythropoietic anemia type IIOpen Targets
0.03Suggestive
acute erythroleukemiaOpen Targets
0.03Suggestive
erythroleukemia, familial, susceptibility toOpen Targets
0.03Suggestive
combined immunodeficiency due to OX40 deficiencyOpen Targets
0.03Suggestive
tuberculosisOpen Targets
0.03Suggestive
uterine fibroidOpen Targets
0.03Suggestive
endometriosisOpen Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PRRG1Shared pathway100%PRRG2Shared pathway100%PROZShared pathway100%PRRG3Shared pathway100%QSER1Protein interaction78%PRSS56Shared pathway67%
Tissue Expression6 tissues
Liver
100%
Bone Marrow
55%
Lung
34%
Brain
8%
Heart
8%
Ovary
2%
Gene Interaction Network
Click a node to explore
PRRG4PRRG1PRRG2PROZPRRG3QSER1PRSS56
PROTEIN STRUCTURE
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AlphaFoldAI-predicted · UniProt Q9BZD6
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.41LoF Tolerant
pLIⓘ
0.04Tolerant
Observed/Expected LoF0.68 [0.35–1.41]
RankingsWhere PRRG4 stands among ~20K protein-coding genes
  • #14,783of 20,598
    Most Researched18
  • #14,579of 17,882
    Most Constrained (LOEUF)1.41
Genes detectedPRRG4
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
The WAGR syndrome gene PRRG4 is a functional homologue of the commissureless axon guidance gene.
PMID: 28859078
PLoS Genet · 2017
1.00
2
PRRG4 promotes breast cancer metastasis through the recruitment of NEDD4 and downregulation of Robo1.
PMID: 33037408
Oncogene · 2020
0.90
3
Cellular localization and characterization of cytosolic binding partners for Gla domain-containing proteins PRRG4 and PRRG2.
PMID: 23873930
J Biol Chem · 2013
0.80
4
Integrated bioinformatics analysis of differences between EAC and ESCC.
PMID: 41162922
BMC Cancer · 2025
0.70
5
Comprehensive Analysis of a Competing Endogenous RNA Co-Expression Network in Chronic Obstructive Pulmonary Disease.
PMID: 37955025
Int J Chron Obstruct Pulmon Dis · 2023
0.60