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10 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
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RASA3
RAS p21 protein activator 3
Chromosome 13 Β· 13q34
NCBI Gene: 22821Ensembl: ENSG00000185989.12HGNC: HGNC:20331UniProt: Q14644
43PubMed Papers
20Diseases
0Drugs
2Pathogenic Variants
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
cytoplasmic side of plasma membraneplatelet activationcalcium channel regulator activityregulation of intracellular signal transductionShort statureneurodegenerative diseaseplacental retentionliver disease
✦AI Summary

RASA3 (RAS p21 protein activator 3) is a GTPase-activating protein that negatively regulates RAS and RAP1 signaling pathways by enhancing their intrinsic GTPase activity 1. The protein functions as a critical gatekeeper in multiple cell types, maintaining cellular quiescence and controlling adhesion dynamics. In T cells, RASA3 suppresses LFA-1-mediated adhesion and is rapidly downregulated upon activation, with loss leading to increased RAP1 activation and impaired lymph node migration 2. RASA3 serves as a negative regulator of T cell activation and adhesion, helping maintain naive T cells in a quiescent state 3. In endothelial cells, RASA3 controls adhesion turnover and vascular lumen integrity through RAP1-dependent mechanisms, with deletion causing embryonic hemorrhages and lethality 4. The protein also regulates platelet function by restraining RAP1 activation in circulating platelets, preventing inappropriate adhesion while allowing rapid activation upon vascular injury 5. Disease relevance includes associations with pulmonary hypertension, where reduced RASA3 expression correlates with increased mortality and disease severity 6. Additionally, RASA3 shows genetic associations with antibody-mediated immune responses to infectious diseases, particularly Epstein-Barr virus 7. Originally identified as an inositol tetrakisphosphate receptor, RASA3 represents a critical regulatory node controlling cellular adhesion and activation across multiple physiological systems 1.

Sources cited
1
RASA3 is a GTPase activating protein targeting Ras and Rap1, originally identified as an inositol tetrakisphosphate receptor
PMID: 25294679
2
RASA3 serves as a gatekeeper limiting T cell activation and adhesion by inactivating RAP1
PMID: 37858490
3
RASA3 suppresses LFA-1 activation in T cells and is rapidly reduced upon T cell activation
PMID: 35857633
4
RASA3 controls endothelial cell adhesion turnover and vascular lumen integrity, with deletion causing embryonic hemorrhages
PMID: 29381707
5
RASA3 restrains RAP1 activation in circulating platelets to maintain quiescence
PMID: 26423530
6
Reduced RASA3 expression associates with pulmonary hypertension risk and increased mortality
PMID: 37101805
7
RASA3 shows genetic associations with antibody-mediated immune responses to infectious diseases
PMID: 33204752
Disease Associationsβ“˜20
Short statureOpen Targets
0.40Weak
neurodegenerative diseaseOpen Targets
0.32Weak
placental retentionOpen Targets
0.28Weak
liver diseaseOpen Targets
0.20Weak
post term pregnancyOpen Targets
0.18Weak
lung cancerOpen Targets
0.18Weak
oral mucosa leukoplakiaOpen Targets
0.17Weak
spinal cord injuryOpen Targets
0.14Weak
ankylosing spondylitisOpen Targets
0.13Weak
eye diseaseOpen Targets
0.12Weak
ThrombocytopeniaOpen Targets
0.09Suggestive
Beta-thalassemia - X-linked thrombocytopeniaOpen Targets
0.09Suggestive
beta-thalassemia-X-linked thrombocytopenia syndromeOpen Targets
0.09Suggestive
macrothrombocytopenia, isolated, 2, autosomal dominantOpen Targets
0.08Suggestive
essential thrombocythemiaOpen Targets
0.08Suggestive
thrombocytopenia 4Open Targets
0.08Suggestive
cataractOpen Targets
0.08Suggestive
trauma complicationOpen Targets
0.08Suggestive
autosomal dominant macrothrombocytopeniaOpen Targets
0.08Suggestive
hepatocellular carcinomaOpen Targets
0.07Suggestive
Pathogenic Variants2
NM_007368.4(RASA3):c.254T>C (p.Val85Ala)Likely pathogenic
Short stature
β˜†β˜†β˜†β˜†2001β†’ Residue 85
NM_007368.4(RASA3):c.892_895del (p.Asp298fs)Pathogenic
Short stature
β˜†β˜†β˜†β˜†2001β†’ Residue 298
View on ClinVar β†—
Related Genes
HRASProtein interaction99%NRASProtein interaction94%MRASProtein interaction71%RAP1AProtein interaction70%RASA1Protein interaction65%RASA2Shared pathway60%
Tissue Expression6 tissues
Bone Marrow
100%
Lung
89%
Brain
65%
Heart
51%
Ovary
31%
Liver
23%
Gene Interaction Network
Click a node to explore
RASA3HRASNRASMRASRAP1ARASA1RASA2
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted Β· UniProt Q14644
View on AlphaFold β†—
Constraintβ“˜
LOEUFβ“˜
0.81LoF Tolerant
pLIβ“˜
0.00Tolerant
Observed/Expected LoF0.66 [0.54–0.81]
RankingsWhere RASA3 stands among ~20K protein-coding genes
  • #9,778of 20,598
    Most Researched43
  • #4,459of 5,498
    Most Pathogenic Variants2
  • #6,849of 17,882
    Most Constrained (LOEUF)0.81
Genes detectedRASA3
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
Mind the GAP: RASA2 and RASA3 GTPase-activating proteins as gatekeepers of T cell activation and adhesion.
PMID: 37858490
Trends Immunol Β· 2023
1.00
2
PMID: 37101805
Pulm Circ Β· 2023
0.90
3
A CRISPR screen targeting PI3K effectors identifies RASA3 as a negative regulator of LFA-1-mediated adhesion in T cells.
PMID: 35857633
Sci Signal Β· 2022
0.80
4
The Ras/Rap GTPase activating protein RASA3: from gene structure to in vivo functions.
PMID: 25294679
Adv Biol Regul Β· 2015
0.70
5
RAP1-GTPase signaling and platelet function.
PMID: 26423530
J Mol Med (Berl) Β· 2016
0.60