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Gene

REST

RE1 silencing transcription factor

Summary

Cytogenetic location
4q12
Coordinates
Chromosome 4: 56,907,900–56,935,844 (GRCh38)
Exons
4 exons across 27.9 kb
Protein
1,097 amino acids
Inheritance
Autosomal dominant
Also known as
NRSF, XBR
Previous symbols
DFNA27
MANE Select transcript
ENST00000309042.12NM_005612.5
Locus type
gene with protein product
  • Very high
  • Confident
  • Low
  • Very low

Reference genomeGRCh38

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

Curated reports relevant to this gene

  • Hereditary Gingival Fibromatosis

    • Autosomal dominant

      Slowly progressive, non-inflammatory overgrowth of the gum tissue, linked to genes such as SOS1 and REST, often starting when the permanent teeth erupt.

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

      Knowing can support an earlier diagnosis and planning of gum surgery and orthodontic care, and inform relatives.

  • Hereditary Wilms Tumour

    • Inherited susceptibility to Wilms tumour (nephroblastoma), including WT1- and REST-related forms.

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

      Regular kidney ultrasound in early childhood allows tumours to be found early; WT1 carriers may also need kidney-function and gonadal checks.

Inheritance

Autosomal dominant
One changed copy, from either parent, is enough. A parent who has one passes it on to each child with a one-in-two chance.

How REST is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
  • Stretches short reads find hard
Chromosome 4, with a bracket where REST sits. REST and 10 genes near it on chromosome 4, from 56,421,872 to 57,421,871.Chromosome 44q12p16.3p16.2p16.1p15.33p15.32p15.31p15.2p15.1p14p13p12p11q11q12q13.1q13.2q13.3q21.1q21.21q21.22q21.23q21.3q22.1q22.2q22.3q23q24q25q26q27q28.1q28.2q28.3q31.1q31.21q31.22q31.23q31.3q32.1q32.2q32.3q33q34.1q34.2q34.3q35.1q35.256.6 Mb56.8 Mb57 Mb57.2 Mb57.4 MbPPAT: 56,393,362–56,436,038, reverse strandPPATPAICS: 56,435,058–56,464,578, forward strandPAICSSRP72: 56,467,591–56,503,891, forward strandSRP72ARL9: 56,505,209–56,524,959, forward strandARL9SPMAP2L: 56,530,601–56,603,556, forward strandSPMAP2LHOPX: 56,647,195–56,681,877, reverse strandHOPXSPINK2: 56,809,857–56,821,869, reverse strandSPINK2REST: 56,907,900–56,935,844, forward strandRESTNOA1: 56,961,620–56,977,625, reverse strandNOA1POLR2B: 56,977,711–57,031,158, forward strandPOLR2BIGFBP7: 56,986,181–57,110,921, reverse strandIGFBP7Diagram of REST: 4 exons on the forward strand of chromosome 4Exon 4: 7 known disease-causing sitesExon 1: 56,907,900–56,908,213, 314 basesExon 2: 56,910,630–56,911,536, 907 basesExon 3: 56,919,787–56,919,870, 84 basesExon 4: 56,929,841–56,935,844, 6,004 bases12345′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 7.3 kb of the gene's 27.9 kb; 3,294 of those bases code for the protein.

How a genome test reads REST

7

positions in REST 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 27.9 kb of REST, introns included, not only the 7.3 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.). REST gene: RE1 silencing transcription factor. Retrieved 17 October 2026, from https://dantelabs.com/genes/rest

Vancouver

Dante Labs. REST gene: RE1 silencing transcription factor [Internet]. [cited 17 October 2026]. Available from: https://dantelabs.com/genes/rest

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