Regulator of Telomere Elongation Helicase 1

Alternative Names

  • RTEL1
  • Novel Helicase-Like Protein
  • NHL
  • Regulator of Telomere Length
  • RTEL
  • Telomere Length Regulator
  • KIAA1088
  • Chromosome 20 Open Reading Frame 41
  • C20ORF41
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OMIM Number

608833

NCBI Gene ID

51750

Uniprot ID

Q9NZ71

Length

38,444 bases

No. of Exons

35

No. of isoforms

8

Protein Name

Regulator of telomere elongation helicase 1

Molecular Mass

133683 Da

Amino Acid Count

1219

Genomic Location

chr20:63,657,810-63,696,253

Gene Map Locus
20q13.33

Description

This gene encodes a DNA helicase which functions in the stability, protection and elongation of telomeres and interacts with proteins in the shelterin complex known to protect telomeres during DNA replication. Mutations in this gene have been associated with dyskeratosis congenita and Hoyerall-Hreidarsson syndrome. Read-through transcription of this gene into the neighboring downstream gene, which encodes tumor necrosis factor receptor superfamily, member 6b, generates a non-coding transcript. Alternative splicing results in multiple transcript variants encoding different isoforms. [From RefSeq]

Epidemiology in the Arab World

View Map
Variant NameCountryGenomic LocationClinvar Clinical SignificanceCTGA Clinical Significance Condition(s)HGVS ExpressionsdbSNPClinvar
NM_001283009.1:c.173C>GUnited Arab EmiratesNC_000020.11:g.63661368C>GUncertain SignificanceNG_033901.1:g.8559C>G; NM_001283009.1:c.173C>G; NP_001269938.1:p.Ala58Gly773669162
NM_001283009.2:c.1993G>A LebanonNC_000020.11:g.63689616G>APathogenic, Uncertain SignificanceNG_033901.1:g.36807G>A; NM_001283009.2:c.1993G>A ; NP_001269938.1:p.Glu665Lys768397005
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