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Gene

RIT1

Ras like without CAAX 1

Summary

Cytogenetic location
1q22
Coordinates
Chromosome 1: 155,897,808–155,911,349 (GRCh38)
Exons
6 exons across 13.5 kb
Protein
219 amino acids
Also known as
RIBB, ROC1, MGC125864, MGC125865
Previous symbols
RIT
MANE Select transcript
ENST00000368323.8NM_006912.6
Locus type
Protein-coding
  • Very high
  • Confident
  • Low
  • Very low

Curated reports relevant to this gene

  • Noonan Syndrome

    • Autosomal dominant

      Noonan Syndrome (NS) is characterized by short stature, typical facial dysmorphism and congenital heart defects.

      • ClinGen rates the link definitive
      • 5 GenCC submitters rate it definitive or strong
      Actionability

      Cardiac surveillance, developmental support, growth hormone therapy, genetic counseling, MEK inhibitor trials

    • Noonan Syndrome and Noonan-Related Syndromes

      A group of RAS pathway conditions, including Noonan, Costello, cardiofaciocutaneous and Legius syndromes, with variable heart defects, short stature and distinctive facial features.

      • ClinGen rates the link definitive
      • 5 GenCC submitters rate it definitive or strong
      Actionability

      Knowing about it supports heart, growth and developmental assessment.

Broader groupings

  • Hypertrophic Cardiomyopathy

    • Autosomal dominant

      One of 62 genes linked to it

      A condition in which the myocardium is hypertrophied without an obvious cause. The hypertrophy is generally asymmetric and may be associated with obstruction of the ventricular outflow tract.

      • ClinGen rates the link definitive
      • 5 GenCC submitters rate it definitive or strong
      Actionability

      Beta-blockers, non-dihydropyridine calcium channel blockers, activity restriction, genetic counseling

How RIT1 is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
  • Stretches short reads find hard
Chromosome 1, with a bracket where RIT1 sits. RIT1 and 29 genes near it on chromosome 1, from 155,404,579 to 156,404,578.Chromosome 11q22p36.33p36.32p36.31p36.23p36.22p36.21p36.13p36.12p36.11p35.3p35.2p35.1p34.3p34.2p34.1p33p32.3p32.2p32.1p31.3p31.2p31.1p22.3p22.2p22.1p21.3p21.2p21.1p13.3p13.2p13.1p12p11.2p11.1q11q12q21.1q21.2q21.3q22q23.1q23.2q23.3q24.1q24.2q24.3q25.1q25.2q25.3q31.1q31.2q31.3q32.1q32.2q32.3q41q42.11q42.12q42.13q42.2q42.3q43q44155.6 Mb155.8 Mb156 Mb156.2 Mb156.4 MbASH1L: 155,335,268–155,563,162, reverse strandASH1LMSTO1: 155,563,235–155,616,644, forward strandMSTO1: 155,626,755–155,648,640, reverse strandYY1AP1: 155,659,446–155,689,334, reverse strandYY1AP1DAP3: 155,687,833–155,740,132, forward strandDAP3GON4L: 155,749,659–155,859,439, reverse strandGON4LSYT11: 155,859,449–155,885,199, forward strandSYT11RIT1: 155,897,808–155,911,349, reverse strandRIT1KHDC4: 155,913,043–155,934,416, reverse strandKHDC4RXFP4: 155,941,638–155,943,087, forward strandRXFP4ARHGEF2: 155,944,685–156,007,070, reverse strandARHGEF2SSR2: 156,009,041–156,020,972, reverse strandSSR2UBQLN4: 156,026,961–156,053,814, reverse strandUBQLN4LAMTOR2: 156,053,916–156,058,817, forward strandLAMTOR2RAB25: 156,061,147–156,070,514, forward strandRAB25MEX3A: 156,072,013–156,082,465, reverse strandMEX3ALMNA: 156,082,572–156,140,085, forward strandLMNASEMA4A: 156,147,366–156,178,869, forward strandSEMA4ASLC25A44: 156,193,932–156,212,796, forward strandSLC25A44PMF1-BGLAP: 156,212,982–156,243,332, forward strandPMF1-BGLAPPMF1: 156,212,989–156,240,585, forward strandPMF1BGLAP: 156,242,154–156,243,317, forward strandBGLAPPAQR6: 156,243,318–156,248,647, reverse strandSMG5: 156,249,223–156,282,862, reverse strandTMEM79: 156,282,928–156,293,185, forward strandTMEM79GLMP: 156,290,088–156,295,699, reverse strandGLMPVHLL: 156,298,624–156,299,307, reverse strandVHLLCCT3: 156,308,792–156,367,873, reverse strandCCT3TSACC: 156,337,314–156,347,019, forward strandTSACCRHBG: 156,369,015–156,385,221, forward strandRHBGDiagram of RIT1: 6 exons on the reverse strand of chromosome 1Exon 5: 16 known disease-causing sitesExon 4: 4 known disease-causing sitesExon 3: 2 known disease-causing sitesExon 2: 4 known disease-causing sitesExon 6: 155,897,808–155,900,618, 2,811 basesExon 5: 155,904,311–155,904,502, 192 basesExon 4: 155,904,731–155,904,804, 74 basesExon 3: 155,910,450–155,910,506, 57 basesExon 2: 155,910,656–155,910,804, 149 basesExon 1: 155,911,243–155,911,349, 107 bases6543215′3′

Exons of the gene's MANE Select transcript (release v1.5), from NCBI and EMBL-EBI, on the GRCh38 reference genome.

Chromosome bands from UCSC's GRCh38 ideogram (2022-10-29).

Nearby genes from Ensembl release 116, on GRCh38.

Exons make up 3.4 kb of the gene's 13.5 kb; 660 of those bases code for the protein.

Sequencing RIT1

26

positions in RIT1 where ClinVar classes a change as pathogenic or likely pathogenic

None of them falls in a region short reads find hard.

A whole genome covers all 13.5 kb of RIT1, introns included, not only the 3.4 kb of exons.

Genes linked to the same conditions

Traits linked to RIT1

The Human Phenotype Ontology lists these as features of the rare conditions RIT1 can cause. Each trait's page lists the other genes linked to it.

External links

Where this comes from

  • HGNC: the gene's symbol, name, other symbols and location.
  • MANE Select v1.5, from NCBI and EMBL-EBI: the transcript, its exons and coding sequence.
  • GenCC and ClinGen, released 2026-10-17: which conditions the gene is linked to, and how strongly.
  • The ACMG secondary findings list, SF v3.2.
  • ClinVar and the GIAB stratifications: known disease-causing sites, and the regions short reads find hard.
  • The Human Phenotype Ontology, release 2026-09-01: the traits linked to the gene. Find out more at hpo.jax.org.
  • Data release 2026.10.17-8666dff2.

Cite this page

APA

Dante Labs. (n.d.). RIT1 gene: Ras like without CAAX 1. Retrieved 17 October 2026, from https://dantelabs.com/genes/rit1

Vancouver

Dante Labs. RIT1 gene: Ras like without CAAX 1 [Internet]. [cited 17 October 2026]. Available from: https://dantelabs.com/genes/rit1

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