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    GENE:

    RIT1 (Ras Like Without CAAX 1)

    i
    Other names: RIT1, Ras Like Without CAAX 1, RIBB, ROC1, RIT, Ras-Like Protein Expressed In Many Tissues, Ric-Like, Expressed In Many Tissues, Ras-Like Without CAAX Protein 1, GTP-Binding Protein Roc1, GTP-Binding Protein Rit1, MGC125864, MGC125865, Ric (Drosophila)-Like, Expressed In Many Tissues, NS8
    14d
    Epithelial Cell-Specific Prognostic Signature (FTH1, RIT1, WASL, NDRG2, KIFC3) Stratifies Cervical Cancer Patients and Correlates With Immune Infiltration. (PubMed, Hum Mutat)
    Crucially, in vitro experiments confirmed that FTH1 knockdown inhibited the proliferation, migration, and invasion of CC cells while enhancing the level of apoptosis in cancer cells. A proposed EpC-specific gene signature for CC may be applicable to support clinical decision-making.
    Journal
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    EGFR (Epidermal growth factor receptor) • RIT1 (Ras Like Without CAAX 1) • LGALS9 (Galectin 9)
    2ms
    Molecular and Clinical Profiles of Patients with RASopathies: Targeted Next-Generation Sequencing Panel Results and Identification of 14 Novel Disease-Causing Variants. (PubMed, Mol Syndromol)
    Accurate clinical correlation and molecular analysis are essential, as different RASopathy syndromes can result from variants in the same genes, while the same syndrome may arise from different genetic alterations. This study identifies novel variants and emphasizes the need for precise diagnostic approaches in these complex disorders.
    Journal • Next-generation sequencing
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    KRAS (KRAS proto-oncogene GTPase) • BRAF (B-raf proto-oncogene) • NRAS (Neuroblastoma RAS viral oncogene homolog) • NF1 (Neurofibromin 1) • PTPN11 (Protein Tyrosine Phosphatase Non-Receptor Type 11) • RIT1 (Ras Like Without CAAX 1)
    2ms
    Venetoclax Added to Fludarabine + Busulfan Prior to Transplant and to Maintenance Therapy for AML, MDS, and MDS/MPN (clinicaltrials.gov)
    P1, N=102, Active, not recruiting, Jacqueline Garcia, MD | Trial completion date: Feb 2026 --> Mar 2027 | Trial primary completion date: Dec 2025 --> May 2026
    Trial completion date • Trial primary completion date
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    KRAS (KRAS proto-oncogene GTPase) • TP53 (Tumor protein P53) • FLT3 (Fms-related tyrosine kinase 3) • ABL1 (ABL proto-oncogene 1) • NRAS (Neuroblastoma RAS viral oncogene homolog) • BCR (BCR Activator Of RhoGEF And GTPase) • BCL2 (B-cell CLL/lymphoma 2) • NPM1 (Nucleophosmin 1) • NF1 (Neurofibromin 1) • RUNX1 (RUNX Family Transcription Factor 1) • SF3B1 (Splicing Factor 3b Subunit 1) • ASXL1 (ASXL Transcriptional Regulator 1) • KMT2A (Lysine Methyltransferase 2A) • PTPN11 (Protein Tyrosine Phosphatase Non-Receptor Type 11) • SRSF2 (Serine and arginine rich splicing factor 2) • BCOR (BCL6 Corepressor) • U2AF1 (U2 Small Nuclear RNA Auxiliary Factor 1) • STAG2 (Stromal Antigen 2) • MECOM (MDS1 And EVI1 Complex Locus) • NUP214 (Nucleoporin 214) • GATA2 (GATA Binding Protein 2) • DEK (DEK Proto-Oncogene) • RIT1 (Ras Like Without CAAX 1) • ZRSR2 (Zinc Finger CCCH-Type, RNA Binding Motif And Serine/Arginine Rich 2)
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    TP53 mutation • KRAS mutation • NRAS mutation • RUNX1 mutation • RAS mutation • ASXL1 mutation • CBL mutation • Chr del(5q)
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    Venclexta (venetoclax) • azacitidine • Inqovi (decitabine/cedazuridine) • fludarabine IV • busulfan
    3ms
    Germline activating sequence variations in RASopathy spectrum genes: genotype-phenotype correlation in a North Indian cohort. (PubMed, Front Genet)
    Our findings underscore the clinical and genetic diversity of RASopathies in the Indian population and highlight the role of next-generation sequencing in early and accurate diagnosis. While exploratory drug-gene interaction analysis provides hypothesis-generating insights, clinical translation requires rigorous validation in functional studies and clinical trials.
    Journal
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    BRAF (B-raf proto-oncogene) • NRAS (Neuroblastoma RAS viral oncogene homolog) • HRAS (Harvey rat sarcoma viral oncogene homolog) • PTPN11 (Protein Tyrosine Phosphatase Non-Receptor Type 11) • MAP2K2 (Mitogen-activated protein kinase kinase 2) • RAF1 (Raf-1 Proto-Oncogene Serine/Threonine Kinase) • MAPK1 (Mitogen-activated protein kinase 1) • RIT1 (Ras Like Without CAAX 1) • LZTR1 (Leucine Zipper Like Transcription Regulator 1)
    4ms
    Ubiquitin-independent pathway regulates the RIT1-MAPK pathway in chordoma progression. (PubMed, Cell Death Dis)
    Mechanistically, REGγ regulates chordoma progression through the ubiquitin- and ATP-independent degradation of RIT1, which modulates the RIT1-MAPK pathway. Inhibition of RIT1 in REGγ-knockdown cells and patient-derived organoids alleviated these effects, suggesting that targeting REGγ may be a promising strategy for chordoma treatment.
    Journal
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    RIT1 (Ras Like Without CAAX 1)
    5ms
    Pathway-specific genomic alterations in pancreatic cancer across diverse cohorts. (PubMed, medRxiv)
    Survival analysis revealed no significant differences in overall survival among H/L patients. However, NHW patients with TP53 pathway alterations exhibited borderline significant differences in survival outcomes.
    Journal • Tumor mutational burden
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    ALK (Anaplastic lymphoma kinase) • TMB (Tumor Mutational Burden) • HRAS (Harvey rat sarcoma viral oncogene homolog) • SMAD4 (SMAD family member 4) • ERBB4 (erb-b2 receptor tyrosine kinase 4) • TGFB1 (Transforming Growth Factor Beta 1) • RIT1 (Ras Like Without CAAX 1) • SMAD2 (SMAD Family Member 2)
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    TP53 mutation • KRAS mutation
    5ms
    Mutant RIT1 cooperates with YAP to drive an EMT-like lung cancer state. (PubMed, Cell Rep)
    Oncogenic cooperation between RIT1M90I and p53/Nf2 loss is driven by synergistic activation of AP-1 transcription factors and can be reversed by the combined inhibition of MEK and TEAD. These data identify YAP/TEAD as a mediator of RIT1's oncogenic capability and nominate TEAD as a potential drug target in RIT1-mutant lung cancer.
    Journal
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    EGFR (Epidermal growth factor receptor) • KRAS (KRAS proto-oncogene GTPase) • NF2 (Neurofibromin 2) • RIT1 (Ras Like Without CAAX 1)
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    KRAS mutation • EGFR mutation • RAS mutation
    5ms
    Structural basis for LZTR1 recognition of RAS GTPases for degradation. (PubMed, Science)
    In cellular and mouse models, mutations disrupting substrate binding phenocopied LZTR1 loss, underscoring its substrate specificity. These findings define RAS recognition mechanisms by LZTR1 and suggest a molecular glue strategy to degrade oncogenic KRAS.
    Journal
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    KRAS (KRAS proto-oncogene GTPase) • RIT1 (Ras Like Without CAAX 1) • LZTR1 (Leucine Zipper Like Transcription Regulator 1)
    6ms
    Another Slice of the Pie Uncovered: RIT1M90I Is an Actionable Driver in Non-Small Cell Lung Cancer. (PubMed, Cancer Res)
    These new RIT1-driven lung cancer models represent valuable tools for the preclinical testing and translation of different therapeutic strategies for this patient population. See related article by Mozzarelli et al., p. 3196 See related article by DiMarco et al., p. 3207.
    Journal
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    RIT1 (Ras Like Without CAAX 1)
    6ms
    Venetoclax Added to Fludarabine + Busulfan Prior to Transplant and to Maintenance Therapy for AML, MDS, and MDS/MPN (clinicaltrials.gov)
    P1, N=102, Active, not recruiting, Jacqueline Garcia, MD | Recruiting --> Active, not recruiting
    Enrollment closed
    |
    KRAS (KRAS proto-oncogene GTPase) • TP53 (Tumor protein P53) • FLT3 (Fms-related tyrosine kinase 3) • ABL1 (ABL proto-oncogene 1) • NRAS (Neuroblastoma RAS viral oncogene homolog) • BCR (BCR Activator Of RhoGEF And GTPase) • BCL2 (B-cell CLL/lymphoma 2) • NPM1 (Nucleophosmin 1) • NF1 (Neurofibromin 1) • RUNX1 (RUNX Family Transcription Factor 1) • SF3B1 (Splicing Factor 3b Subunit 1) • ASXL1 (ASXL Transcriptional Regulator 1) • KMT2A (Lysine Methyltransferase 2A) • PTPN11 (Protein Tyrosine Phosphatase Non-Receptor Type 11) • SRSF2 (Serine and arginine rich splicing factor 2) • BCOR (BCL6 Corepressor) • U2AF1 (U2 Small Nuclear RNA Auxiliary Factor 1) • STAG2 (Stromal Antigen 2) • MECOM (MDS1 And EVI1 Complex Locus) • NUP214 (Nucleoporin 214) • GATA2 (GATA Binding Protein 2) • DEK (DEK Proto-Oncogene) • RIT1 (Ras Like Without CAAX 1) • ZRSR2 (Zinc Finger CCCH-Type, RNA Binding Motif And Serine/Arginine Rich 2)
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    TP53 mutation • KRAS mutation • NRAS mutation • RUNX1 mutation • RAS mutation • ASXL1 mutation • CBL mutation • Chr del(5q)
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    Venclexta (venetoclax) • azacitidine • Inqovi (decitabine/cedazuridine) • fludarabine IV • busulfan
    6ms
    Pathway-Specific Genomic Alterations in Pancreatic Cancer Across Populations at Risk. (PubMed, Int J Mol Sci)
    This study reveals ethnicity-specific pathway alterations in PC, with SMAD2, ERBB4, ALK, and CTNNB1 mutations being more frequent in H/L patients, while SMAD4 and PI3K alterations had prognostic value in NHW patients. These findings indicate the importance of incorporating ethnicity-specific molecular profiling into precision oncology for PC.
    Journal
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    ALK (Anaplastic lymphoma kinase) • TP53 (Tumor protein P53) • HRAS (Harvey rat sarcoma viral oncogene homolog) • SMAD4 (SMAD family member 4) • CTNNB1 (Catenin (cadherin-associated protein), beta 1) • ERBB4 (erb-b2 receptor tyrosine kinase 4) • TGFB1 (Transforming Growth Factor Beta 1) • RIT1 (Ras Like Without CAAX 1) • SMAD2 (SMAD Family Member 2)
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    TP53 mutation • KRAS mutation • ALK mutation
    6ms
    Mirror syndrome and placental ectopic liver in association with de novo SOS1 variant. (PubMed, Eur J Med Genet)
    Our findings confirm that mirror syndrome is a potential manifestation of Rasopathies, while the role of ectopic liver tissue in the placenta remains uncertain. Future research should focus on genetic factors underlying mirror syndrome rather than incidental placental anomalies.
    Journal
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    RIT1 (Ras Like Without CAAX 1)