Skip to main content
. Author manuscript; available in PMC: 2024 Dec 11.
Published in final edited form as: Nat Rev Genet. 2022 Oct 31;24(4):211–234. doi: 10.1038/s41576-022-00539-9

Table 1 |.

DNA helicases and non-B DNA structures

Helicase Cellular function Reported types of non-B DNA processed Results from helicase deficiency Ref.
Superfamily 1 DNA helicases
PIF1 Unwinding replication barriers, assisting fork progression G4 Genetic instability, increased risk of cancer 289
RRM3 Unwinding replication barriers, assisting fork progression G4 Genetic instability, increased risk of cancer 290
DNA2 Telomere maintenance; helicase and G4 nuclease G4 Cell senescence, telomere replication defects, genetic instability, increased risk of cancer 148
Srs2 Post-replication repair to UV, ionizing radiation or MMS lesions Cruciform at inverted repeats, hairpin at triplet repeats UV sensitivity, genetic instability 291
UvrD Nucleotide excision repair, mismatch repair, HR G4, cruciform/Holliday junctions, triplet repeats UV sensitivity, genetic instability 292
Rep DNA replication G4 Slow progression of chromosomal replication forks 293
RecBCD Helicase and nuclease activities, DSB repair by HR Cruciform at inverted repeat, hairpin at triplet repeat Genetic instability 294
Superfamily 2 DNA helicases
RECQ1 (also known as RECQL and RECQL1) DNA repair, cell cycle and growth, telomere maintenance, transcription Cruciform/Holliday junctions, very weak on G4 Genetic instability and increased risk of cancer 295
RECQ2 (also known as BLM) DNA replication, immunoglobulin class-switch recombination G4, triplex H-DNA, direct repeats including triplet repeats Hereditary Bloom syndrome: primordial dwarfism, genetic instability, increased risk of cancer 296
Him-6 BLM homologue in Caenorhabditis elegans G4 Genetic instability 297
RECQ3 (also known as WRN) Telomere maintenance G4, hairpin at triplet repeat, triplex H-DNA, Z-DNA Hereditary Werner syndrome, premature ageing, increased risk of cancer, cell senescence 298
Sgs1 Yeast homologue of human BLM and WRN. Forms a complex with Top3 and Rmi1. DNA replication, regulating HR Cruciform/palindrome, G4, hairpin at triplet repeat Genetic instability 291
RECQ4 (also known as RTS) ATPase activity and single-strand annealing activity, replisome assembly Holliday junctions, G4 Hereditary Rothmund–Thomson, RAPADILINO and Baller–Gerold syndromes: skin, hair, skeletal and dental abnormalities, increased risk of cancer 299
RECQ5 DNA replication, transcription, repair, suppressing sister chromatid exchanges during HR G4, an order of magnitude weaker than BLM and WRN Genetic instability 300
RTEL1 Telomere maintenance and HR regulation G4, hairpin at triplet repeat Hoyeraal–Hreidarsson syndrome, pulmonary fibrosis and/or bone marrow failure, telomere-related 3 (PFBMFT3), dyskeratosis congenita, autosomal recessive 5 (DKCB5) 301
DHX36 RNA and DNA helicase activity, transcription and translation regulation, genetic stability, telomere maintenance G4 Genetic instability and increased risk of cancer 302
DHX9 DNA replication, transcription, translation, microRNA biogenesis, genetic stability Triplex H-DNA, Z-DNA Genetic instability and increased risk of cancer 303
FANCJ DNA repair, HR, replication fork progress during replication stress G4, hairpin at triplet repeat Hereditary breast and ovarian cancer, Fanconi anaemia, bone marrow failure 304
Dog-1 FANCJ homologue in C. elegans G4 Genetic instability 305
XPB/XPD TFIIH components with functions in nucleotide excision repair G4, triplex H-DNA Xeroderma pigmentosum (UV sensitivity, cancer); trichothiodystrophy, Cockayne syndrome (development) 306
DDX5 DNA and RNA helicase activity, transcriptional regulation, splicing G4 Aberrantly expressed in many tumours 307
DDX11 (also known as CHLR1), CHL1 in yeast Chromosome segregation, cell cycle progression, sister chromatid cohesion, putative RNA helicase, translation initiation, splicing G4, triplex H-DNA Warsaw breakage syndrome 308
ATRX Chromosome alignment and meiotic spindle organization, recombination pathway selection G4 α-Thalassaemia with mental retardation 309

DSB, double-strand break; HR, homologous recombination; MMS, methyl methanesulfonate.