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Gene

RNF213

ring finger protein 213

Summary

Cytogenetic location
17q25.3
Coordinates
Chromosome 17: 80,260,852–80,398,794 (GRCh38)
Exons
68 exons across 137.9 kb
Protein
5,207 amino acids
Also known as
KIAA1554, NET57, ALO17
Previous symbols
C17orf27, KIAA1618, MYMY2
MANE Select transcript
ENST00000582970.6NM_001256071.3
Locus type
gene with protein product

Reference genomeGRCh38

Positions on gene and variant pages follow this choice, in this browser.

Curated report relevant to this gene

  • Moyamoya Disease

    • Progressive narrowing of the arteries at the base of the brain, linked to changes in RNF213 (a susceptibility gene with reduced penetrance, especially common in East Asian populations), ACTA2 or GUCY1A1, which can cause strokes or mini-strokes.

      • 3 GenCC submitters rate it definitive or strong
      What knowing changes

      Knowing about it supports early assessment of symptoms such as transient weakness, and access to specialist stroke prevention and surgical treatment.

How RNF213 is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
  • Stretches short reads find hard
Chromosome 17, with a bracket where RNF213 sits. RNF213 and 12 genes near it on chromosome 17, from 79,829,823 to 80,829,822.Chromosome 1717q25.3p13.3p13.2p13.1p12p11.2p11.1q11.1q11.2q12q21.1q21.2q21.31q21.32q21.33q22q23.1q23.2q23.3q24.1q24.2q24.3q25.1q25.2q25.380 Mb80.2 Mb80.4 Mb80.6 Mb80.8 MbCBX4: 79,833,156–79,839,486, reverse strandCBX4TBC1D16: 79,932,343–80,035,887, reverse strandTBC1D16CCDC40: 80,036,000–80,100,613, forward strandCCDC40GAA: 80,101,526–80,119,881, forward strandGAAEIF4A3: 80,132,700–80,147,151, reverse strandEIF4A3CARD14: 80,169,992–80,216,073, forward strandCARD14SGSH: 80,206,716–80,220,928, reverse strandSGSHSLC26A11: 80,219,699–80,253,509, forward strandSLC26A11RNF213: 80,260,852–80,398,794, forward strandRNF213ENDOV: 80,415,147–80,438,086, forward strandENDOVNPTX1: 80,466,834–80,477,843, reverse strandNPTX1RPTOR: 80,544,819–80,966,371, forward strandRPTOR: 80,545,075–80,545,134, forward strandDiagram of RNF213: 68 exons on the forward strand of chromosome 17Exon 9: 1 known disease-causing siteExon 16: 1 known disease-causing siteExon 27: 1 known disease-causing siteExon 37: 1 known disease-causing siteExon 44: 3 known disease-causing sitesExon 46: 4 known disease-causing sitesExon 48: 1 known disease-causing siteExon 1: 80,260,852–80,260,902, 51 basesExon 2: 80,263,574–80,263,778, 205 basesExon 3: 80,273,241–80,273,404, 164 basesExon 4: 80,287,815–80,288,363, 549 basesExon 5: 80,288,633–80,288,755, 123 basesExon 6: 80,289,659–80,289,837, 179 basesExon 7: 80,290,570–80,290,728, 159 basesExon 8: 80,291,628–80,291,827, 200 basesExon 9: 80,294,720–80,295,003, 284 basesExon 10: 80,295,557–80,295,813, 257 basesExon 11: 80,298,321–80,298,518, 198 basesExon 12: 80,306,252–80,306,468, 217 basesExon 13: 80,307,128–80,307,201, 74 basesExon 14: 80,309,018–80,309,171, 154 basesExon 15: 80,313,012–80,313,167, 156 basesExon 16: 80,317,188–80,317,277, 90 basesExon 17: 80,319,190–80,319,312, 123 basesExon 18: 80,325,030–80,325,198, 169 basesExon 19: 80,327,816–80,327,989, 174 basesExon 20: 80,328,328–80,328,477, 150 basesExon 21: 80,332,006–80,332,631, 626 basesExon 22: 80,334,105–80,334,270, 166 basesExon 23: 80,336,161–80,336,378, 218 basesExon 24: 80,337,586–80,337,726, 141 basesExon 25: 80,337,833–80,337,997, 165 basesExon 26: 80,339,201–80,340,356, 1,156 basesExon 27: 80,343,132–80,343,325, 194 basesExon 28: 80,343,857–80,344,015, 159 basesExon 29: 80,344,678–80,348,286, 3,609 basesExon 30: 80,349,770–80,349,906, 137 basesExon 31: 80,350,301–80,350,396, 96 basesExon 32: 80,351,685–80,351,803, 119 basesExon 33: 80,352,940–80,353,059, 120 basesExon 34: 80,353,512–80,353,666, 155 basesExon 35: 80,354,019–80,354,166, 148 basesExon 36: 80,354,441–80,354,576, 136 basesExon 37: 80,358,288–80,358,479, 192 basesExon 38: 80,360,061–80,360,206, 146 basesExon 39: 80,361,734–80,361,888, 155 basesExon 40: 80,363,102–80,363,314, 213 basesExon 41: 80,363,609–80,363,790, 182 basesExon 42: 80,364,433–80,364,553, 121 basesExon 43: 80,367,748–80,367,848, 101 basesExon 44: 80,367,961–80,368,143, 183 basesExon 45: 80,369,502–80,369,671, 170 basesExon 46: 80,369,768–80,369,867, 100 basesExon 47: 80,371,874–80,371,985, 112 basesExon 48: 80,372,521–80,372,734, 214 basesExon 49: 80,372,975–80,373,165, 191 basesExon 50: 80,374,458–80,374,589, 132 basesExon 51: 80,375,760–80,375,870, 111 basesExon 52: 80,376,301–80,376,543, 243 basesExon 53: 80,376,882–80,376,963, 82 basesExon 54: 80,377,762–80,377,796, 35 basesExon 55: 80,379,620–80,379,714, 95 basesExon 56: 80,380,831–80,380,987, 157 basesExon 57: 80,381,547–80,381,727, 181 basesExon 58: 80,382,979–80,383,070, 92 basesExon 59: 80,383,677–80,383,928, 252 basesExon 60: 80,385,039–80,385,171, 133 basesExon 61: 80,385,538–80,385,621, 84 basesExon 62: 80,386,250–80,386,430, 181 basesExon 63: 80,386,690–80,386,891, 202 basesExon 64: 80,388,612–80,388,689, 78 basesExon 65: 80,389,173–80,389,367, 195 basesExon 66: 80,389,828–80,389,917, 90 basesExon 67: 80,390,012–80,390,196, 185 basesExon 68: 80,393,345–80,398,794, 5,450 bases15101520253035404550556065685′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 21.1 kb of the gene's 137.9 kb; 15,624 of those bases code for the protein.

How a genome test reads RNF213

13

positions in RNF213 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 137.9 kb of RNF213, introns included, not only the 21.1 kb of exons.

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 2026-10-16: 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 2026.10.16-c6eea6bf.

Cite this page

APA

Dante Labs. (n.d.). RNF213 gene: ring finger protein 213. Retrieved 17 October 2026, from https://dantelabs.com/genes/rnf213

Vancouver

Dante Labs. RNF213 gene: ring finger protein 213 [Internet]. [cited 17 October 2026]. Available from: https://dantelabs.com/genes/rnf213

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