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Target intelligence / Profile preview
Ras-related GTP-binding protein C (RRAGC)
- Target
- RRAGC
- Molecular classification
- Small GTPase, G protein, Guanine nucleotide-binding protein, GTR/RAG GTP-binding protein family
01
Overview
Ras-related GTP-binding protein C (RRAGC) is a monomeric small GTPase that acts as a metabolic sensor, controlling the cellular response to amino acid availability via regulation of the mTORC1 signaling complex. RRAGC functions by forming heterodimeric complexes (such as RagA–RagC or RagB–RagC), switching between GTP- and GDP-bound states to facilitate the recruitment and activation of mTORC1 at lysosomal membranes. Proper activity of RRAGC is essential for mTORC1-mediated control of cell growth, metabolism, and autophagy. Pathogenic mutations in RRAGC disrupt these processes and are linked to rare syndromic diseases and cancer, making RRAGC a target of therapeutic interest, particularly for mTOR inhibitors in cases with aberrant pathway activation.
- Other names
- Rag CRagCGTPase-interacting protein 2TIB929GTR2FLJ13311LNGODSLNGOSRAGC
02
Mechanism of action
mTORC1 inhibition (via direct or indirect targeting due to hyperactivation caused by RRAGC mutations, e.g., by rapamycin)
03
Biological functions
Regulation of mTORC1 signalingCellular response to amino acid availabilityProtein heterodimerization (forms heterodimers with RagA/B)Signal transduction
04
Disease associations
Long-Olsen-Distelmaier syndrome (syndromic dilated cardiomyopathy, developmental and organ defects)Birt-Hogg-Dubé syndromeCancer (notably follicular lymphoma with activating RRAGC mutations)
05
Safety considerations
mTORC1 pathway modulation can affect immunity, metabolism, and growth, leading to potential risks (infections, metabolic disturbances) when using mTOR inhibitors for RRAGC-driven diseaseHyperactive mTORC1 signaling can contribute to cardiomyopathy, cancer, metabolic and developmental syndromes
06
Interacting drugs
Rapamycin (and related mTOR inhibitors, proposed for patients with hyperactive mTORC1 signaling due to RRAGC mutations)
07
Biomarkers
RRAGC mutation status (for diagnostic or prognostic relevance in syndromic cardiomyopathy, certain cancers)mTORC1 activity (indirect, as a downstream biomarker for RRAGC pathway activity)
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