UniProt:Q9HB90 RRAGC

chain
  • initiator methionine:1
  • chain:2-399
checksum 35B3171253CC69CF
comment
  • FUNCTION Guanine nucleotide-binding protein that plays a crucial role in the cellular response to amino acid availability through regulation of the mTORC1 signaling cascade (PubMed:20381137, PubMed:24095279, PubMed:27234373, PubMed:31601708, PubMed:31601764, PubMed:32612235, PubMed:34071043, PubMed:36697823, PubMed:37057673). Forms heterodimeric Rag complexes with RagA/RRAGA or RagB/RRAGB and cycles between an inactive GTP-bound and an active GDP-bound form: RagC/RRAGC is in its active form when GDP-bound RagC/RRAGC forms a complex with GTP-bound RagA/RRAGA (or RagB/RRAGB) and in an inactive form when GTP-bound RagC/RRAGC heterodimerizes with GDP-bound RagA/RRAGA (or RagB/RRAGB) (PubMed:24095279, PubMed:31601708, PubMed:31601764, PubMed:32868926). In its GDP-bound active form, promotes the recruitment of mTORC1 to the lysosomes and its subsequent activation by the GTPase RHEB (PubMed:20381137, PubMed:24095279, PubMed:27234373, PubMed:32612235, PubMed:36697823). This is a crucial step in the activation of the MTOR signaling cascade by amino acids (PubMed:20381137, PubMed:24095279, PubMed:27234373). Also plays a central role in the non-canonical mTORC1 complex, which acts independently of RHEB and specifically mediates phosphorylation of MiT/TFE factors TFEB and TFE3: GDP-bound RagC/RRAGC mediates recruitment of MiT/TFE factors TFEB and TFE3 (PubMed:32612235, PubMed:36697823).CATALYTIC ACTIVITY GTP + H2O = GDP + phosphate + H(+)ACTIVITY REGULATION The activation of RagC/RRAGC is mediated by a GTPase activating protein (GAP) (PubMed:24095279). In high-amino acid conditions, activated by GTPase activating protein FLCN that stimulates RRAGC GTPase activity to turn it into its active GDP-bound form (PubMed:24095279). In response to amino acid depletion, the GATOR1 complex inactivates RagC/RRAGC by securing the GTP-bound inactive form (PubMed:35338845).SUBUNIT Forms a heterodimer with RRAGA, in a sequence-independent manner, and RRAGB (PubMed:11073942, PubMed:14660641, PubMed:32868926). Heterodimerization stabilizes proteins of the heterodimer (PubMed:11073942). The GDP-bound form of RRAGC (in complex with the GTP-bound form of RRAGA or RRAGB), interacts with RPTOR, thereby promoting recruitment of mTORC1 to the lysosomes (PubMed:18497260, PubMed:31601708, PubMed:31601764). Component of the lysosomal folliculin complex (LFC), composed of FLCN, FNIP1 (or FNIP2), RagA/RRAGA or RagB/RRAGB GDP-bound, RagC/RRAGC or RagD/RRAGD GTP-bound, and Ragulator (PubMed:31672913, PubMed:31704029, PubMed:32868926). Interacts with NOL8 (PubMed:14660641). Interacts with SH3BP4; the interaction with this negative regulator is most probably direct, preferentially occurs with the inactive GDP-bound form of RRAGB, is negatively regulated by amino acids and prevents interaction with RPTOR (PubMed:22575674). The Rag heterodimer interacts with SLC38A9; the probable amino acid sensor (PubMed:25561175, PubMed:25567906, PubMed:32868926). Interacts with SESN1, SESN2 and SESN3 (PubMed:25259925). Interacts with PIP4P1 (By similarity). The Rag heterodimer interacts with the Ragulator complex (PubMed:32868926). The GDP-bound form interacts with TFEB (PubMed:32612235, PubMed:34071043, PubMed:36697823, PubMed:37057673). The GDP-bound form interacts with TFE3 (By similarity).INTERACTION Predominantly cytoplasmic (PubMed:11073942). Recruited to the lysosome surface by the Ragulator complex (PubMed:20381137, PubMed:28935770). May shuttle between the cytoplasm and nucleus, depending on the bound nucleotide state of associated RRAGA (PubMed:11073942).DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE RRAGC mutations have been found in a patient with idiopathic dilated cardiomyopathy with ventricular dilation and systolic dysfunction, bilateral cataracts, and mild facial dysmorphisms.SIMILARITY Belongs to the GTR/RAG GTP-binding protein family.SEQUENCE CAUTION Truncated N-terminus.
created [InstanceEdit:217385] Schmidt, EE, 2008-03-27 06:23:53
crossReference
databaseName UniProt
dbId 246466
description
  • recommendedName: Ras-related GTP-binding protein C shortName: Rag C shortName: RagC ecNumber evidence="3 26"3.6.5.- alternativeName: GTPase-interacting protein 2 alternativeName: TIB929
displayName UniProt:Q9HB90 RRAGC
geneName
  • RRAGC
identifier Q9HB90
isSequenceChanged false
keyword
  • 3D-structure
  • Acetylation
  • Cardiomyopathy
  • Cytoplasm
  • Disease variant
  • GTP-binding
  • Hydrolase
  • Lysosome
  • Membrane
  • Nucleotide-binding
  • Nucleus
  • Phosphoprotein
  • Proteomics identification
  • Reference proteome
modified [InstanceEdit:9963647] Weiser, Joel, 2025-08-15
moleculeType Protein
name
  • RRAGC
otherIdentifier
  • 11719397_a_at
  • 11719398_s_at
  • 11751353_a_at
  • 11758809_at
  • 16685596
  • 218088_PM_s_at
  • 218088_s_at
  • 222514_PM_at
  • 222514_at
  • 2407728
  • 2407730
  • 2407731
  • 2407732
  • 2407733
  • 2407734
  • 2407735
  • 2407736
  • 2407738
  • 2407739
  • 2407740
  • 2407741
  • 2407742
  • 2407743
  • 2407744
  • 2407746
  • 2407747
  • 2407748
  • 2407749
  • 2407750
  • 2407751
  • 2407752
  • 242531_PM_at
  • 242531_at
  • 42342_r_at
  • 52905_at
  • 63799_at
  • 64121
  • 7915160
  • A_23_P97621
  • A_23_P97623
  • GE54855
  • GE80280
  • GO:0000045
  • GO:0000166
  • GO:0000287
  • GO:0002181
  • GO:0003924
  • GO:0005515
  • GO:0005525
  • GO:0005634
  • GO:0005654
  • GO:0005737
  • GO:0005764
  • GO:0005765
  • GO:0005829
  • GO:0006351
  • GO:0006915
  • GO:0007040
  • GO:0007264
  • GO:0008104
  • GO:0008380
  • GO:0009267
  • GO:0010507
  • GO:0016020
  • GO:0016787
  • GO:0019003
  • GO:0030163
  • GO:0031146
  • GO:0031648
  • GO:0031669
  • GO:0032006
  • GO:0034198
  • GO:0038202
  • GO:0043200
  • GO:0043495
  • GO:0045947
  • GO:0045948
  • GO:0046982
  • GO:0051020
  • GO:0060090
  • GO:0061462
  • GO:0071230
  • GO:0072657
  • GO:0140767
  • GO:1900181
  • GO:1903432
  • GO:1904263
  • GO:1990131
  • GO:1990877
  • HMNXSV003030869
  • Hs.108144.0.A1_3p_at
  • Hs.110950.0.A2_3p_at
  • ILMN_1760121
  • PH_hs_0006103
  • TC01006351.hg
  • g11995471_3p_a_at
physicalEntity
referenceDatabase [ReferenceDatabase:2] UniProt
referenceGene
referenceTranscript
schemaClass ReferenceGeneProduct
secondaryIdentifier
  • RRAGC_HUMAN
  • Q9H202
  • Q9H8Q8
sequenceLength 399
species [Species:48887] Homo sapiens
stId uniprot:Q9HB90
url http://purl.uniprot.org/uniprot/Q9HB90
Cite Us!