The Chromatin Remodeler HELLS: A New Regulator in DNA Repair, Genome Maintenance, and Cancer

Estanislao Peixoto, Asad Khan, Zachary A. Lewis, Rafael Contreras-Galindo, Wioletta Czaja

Research output: Contribution to journalReview articlepeer-review

4 Scopus citations


Robust, tightly regulated DNA repair is critical to maintaining genome stability and preventing cancer. Eukaryotic DNA is packaged into chromatin, which has a profound, yet incompletely understood, regulatory influence on DNA repair and genome stability. The chromatin remodeler HELLS (helicase, lymphoid specific) has emerged as an important epigenetic regulator of DNA repair, genome stability, and multiple cancer-associated pathways. HELLS belongs to a subfamily of the conserved SNF2 ATP-dependent chromatin-remodeling complexes, which use energy from ATP hydrolysis to alter nucleosome structure and packaging of chromatin during the processes of DNA replication, transcription, and repair. The mouse homologue, LSH (lymphoid-specific helicase), plays an important role in the maintenance of heterochromatin and genome-wide DNA methylation, and is crucial in embryonic development, gametogenesis, and maturation of the immune system. Human HELLS is abundantly expressed in highly proliferating cells of the lymphoid tissue, skin, germ cells, and embryonic stem cells. Mutations in HELLS cause the human immunodeficiency syndrome ICF (Immunodeficiency, Centromeric instability, Facial anomalies). HELLS has been implicated in many types of cancer, including retinoblastoma, colorectal cancer, hepatocellular carcinoma, and glioblastoma. Here, we review and summarize accumulating evidence highlighting important roles for HELLS in DNA repair, genome maintenance, and key pathways relevant to cancer development, progression, and treatment.

Original languageEnglish (US)
Article number9313
JournalInternational journal of molecular sciences
Issue number16
StatePublished - Aug 2022

Bibliographical note

Funding Information:
This work was funded by NIH/NIGMS, grant number R01GM143428 to Czaja, W.

Publisher Copyright:
© 2022 by the authors.


  • ATP-dependent chromatin remodelers
  • DNA damage
  • DNA repair
  • LSH
  • cancer
  • heterochromatin

PubMed: MeSH publication types

  • Journal Article
  • Review


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