AI Article Synopsis

  • Most rare diseases are genetic, but 60% of patients still lack a diagnosis due to limited understanding of the human genome, particularly transposable elements (TEs), which make up about 50% of it.
  • This study aims to bridge the knowledge gap by analyzing TE insertions in Swedish individuals and comparing them to a global dataset, creating seven databases that show many TEs are common across populations.
  • The research highlights that while rare TE insertions can affect protein-coding genes, they infrequently impact known disease-causing genes and demonstrates successful identification of disease-causing TEs in clinical cases, underlining their significance in rare disease diagnostics.

Article Abstract

The majority of rare diseases are genetic, and regardless of advanced high-throughput genomics-based investigations, 60% of patients remain undiagnosed. A major factor limiting our ability to identify disease-causing alterations is a poor understanding of the morbid and normal human genome. A major genomic contributor of which function and distribution remain largely unstudied are the transposable elements (TE), which constitute 50% of our genome. Here we aim to resolve this knowledge gap and increase the diagnostic yield of rare disease patients investigated with clinical genome sequencing. To this end we characterized TE insertions in 1000 Swedish individuals from the SweGen dataset and 2504 individuals from the 1000 Genomes Project (1KGP), creating seven population-specific TE insertion databases. Of note, 66% of TE insertions in SweGen were present at >1% in the 1KGP databases, proving that most insertions are common across populations. Focusing on the rare TE insertions, we show that even though ~0.7% of those insertions affect protein coding genes, they rarely affect known disease casing genes (<0.1%). Finally, we applied a TE insertion identification workflow on two clinical cases where disease causing TE insertions were suspected and could verify the presence of pathogenic TE insertions in both. Altogether we demonstrate the importance of TE insertion detection and highlight possible clinical implications in rare disease diagnostics.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10381067PMC
http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0289346PLOS

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