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Wild-type C9orf72 expression is a genetic modifier of C9-ALS survival - PubMed

4 hours ago
  • #C9orf72
  • #ALS
  • #genetic modifiers
  • Amyotrophic lateral sclerosis (ALS) is highly heritable but lacks identifiable genetic causes in most cases.
  • A multi-omic QTL atlas was created using 594 human motor neuron lines (522 ALS patients, 72 controls).
  • Common and rare variants on the wild-type C9orf72 allele form regulatory haplotypes influencing expression levels.
  • C9-ALS patients were stratified into four subgroups with different survival trajectories based on C9orf72 expression.
  • Ultra-rare intronic variants create cryptic exons and structural variants in ALS genes, aiding in molecular diagnosis.
  • QTL mapping in patient-derived motor neurons reveals regulatory modifiers and hidden pathogenic events in ALS.