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Gene

RNF168

ring finger protein 168

Summary

Cytogenetic location
3q29
Coordinates
Chromosome 3: 196,468,783–196,503,768 (GRCh38)
Exons
6 exons across 35 kb
Protein
571 amino acids
Also known as
FLJ35794
MANE Select transcript
ENST00000318037.3NM_152617.4
Locus type
Protein-coding
  • Very high
  • Confident
  • Low
  • Very low

Curated report relevant to this gene

    • Autosomal Recessive Cerebellar Ataxias (incl. CANVAS and AVED)

      • Autosomal Recessive Cerebellar AtaxiaAutosomal recessive

        One of 57 genes linked to it

        A group of recessively inherited ataxias caused by biallelic variants in genes such as RFC1, FXN, SACS, TTPA, SETX, APTX, PNKP, ANO10 and SYNE1.

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

        Ataxia with vitamin E deficiency (TTPA) is treatable with vitamin E supplements; other forms are managed with physiotherapy and symptom care, and relatives can be offered carrier testing.

    How RNF168 is laid out

    • Coding sequence
    • Untranslated region
    • Intron
    • Direction of transcription
    • Known disease-causing sites
    • Stretches short reads find hard
    Chromosome 3, with a bracket where RNF168 sits. RNF168 and 21 genes near it on chromosome 3, from 195,986,276 to 196,986,275.Chromosome 33q29p26.3p26.2p26.1p25.3p25.2p25.1p24.3p24.2p24.1p23p22.3p22.2p22.1p21.33p21.32p21.31p21.2p21.1p14.3p14.2p14.1p13p12.3p12.2p12.1p11.2p11.1q11.1q11.2q12.1q12.2q12.3q13.11q13.12q13.13q13.2q13.31q13.32q13.33q21.1q21.2q21.3q22.1q22.2q22.3q23q24q25.1q25.2q25.31q25.32q25.33q26.1q26.2q26.31q26.32q26.33q27.1q27.2q27.3q28q29196 Mb196.2 Mb196.4 Mb196.6 Mb196.8 MbTFRC: 196,012,511–196,082,162, reverse strandTFRCZDHHC19: 196,197,452–196,211,437, reverse strandZDHHC19SLC51A: 196,211,487–196,244,766, forward strandSLC51APCYT1A: 196,214,222–196,287,957, reverse strandPCYT1A: 196,248,230–196,318,222, reverse strandDYNLT2B: 196,291,219–196,318,328, reverse strandDYNLT2BTM4SF19-DYNLT2B: 196,316,082–196,338,373, reverse strandTM4SF19-DYNLT2BTM4SF19: 196,319,342–196,338,604, reverse strandTM4SF19UBXN7: 196,347,659–196,432,476, reverse strandUBXN7RNF168: 196,468,783–196,503,768, reverse strandRNF168SMCO1: 196,506,877–196,515,368, reverse strandSMCO1WDR53: 196,552,217–196,569,263, reverse strandWDR53FBXO45: 196,568,611–196,589,059, forward strandFBXO45NRROS: 196,639,676–196,707,522, forward strandNRROSPIGX: 196,639,775–196,736,016, forward strandPIGXCEP19: 196,706,268–196,712,603, reverse strandCEP19PAK2: 196,739,743–196,832,647, forward strandPAK2SENP5: 196,867,856–196,934,718, forward strandSENP5NCBP2: 196,935,402–196,942,594, reverse strandNCBP2NCBP2AS2: 196,942,674–196,943,543, forward strandNCBP2AS2PIGZ: 196,946,344–196,969,060, reverse strandPIGZMELTF: 196,980,590–197,029,874, reverse strandMELTFDiagram of RNF168: 6 exons on the reverse strand of chromosome 3Exon 6: 2 known disease-causing sitesExon 4: 2 known disease-causing sitesExon 3: 3 known disease-causing sitesExon 1: 1 known disease-causing siteExon 6: 196,468,783–196,472,772, 3,990 basesExon 5: 196,475,231–196,475,312, 82 basesExon 4: 196,483,770–196,483,891, 122 basesExon 3: 196,487,399–196,487,578, 180 basesExon 2: 196,488,607–196,488,683, 77 basesExon 1: 196,502,873–196,503,768, 896 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 5.3 kb of the gene's 35 kb; 1,716 of those bases code for the protein.

    Sequencing RNF168

    6Low

    How hard RNF168 is to sequence with short reads, out of 100

    • Mappability7.3%
    • Homology0%
    • Repeats17%
    • GC content7.9%
    • Reference coverage0%

    8

    positions in RNF168 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 35 kb of RNF168, introns included, not only the 5.3 kb of exons.

    Genes linked to the same conditions

    Traits linked to RNF168

    The Human Phenotype Ontology lists these as features of the rare conditions RNF168 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 Genome in a Bottle genome stratifications, from NIST: the sequencing difficulty score.
    • 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.). RNF168 gene: ring finger protein 168. Retrieved 17 October 2026, from https://dantelabs.com/genes/rnf168

    Vancouver

    Dante Labs. RNF168 gene: ring finger protein 168 [Internet]. [cited 17 October 2026]. Available from: https://dantelabs.com/genes/rnf168

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