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KIF5A and ALS: a clinical and genetic description of a case series and review of literature - PubMed

5 hours ago
  • #genetics
  • #ALS
  • #KIF5A
  • Approximately 10% of ALS cases are familial, while 90% are sporadic.
  • KIF5A was identified as a novel ALS-associated gene in 2018.
  • A study of 224 patients with motor neuron disease (MND) found eight carrying KIF5A variants.
  • Among the eight patients, four had familial ALS and four were sporadic cases.
  • 75% of the KIF5A variant carriers were female, with a mean age of onset at 59 years.
  • Disease progression varied, with five patients being slow-progressors and three fast-progressors.
  • Three of the seven KIF5A variants identified were previously undescribed in literature.
  • Two patients had KIF5A variants alongside mutations in other ALS genes, including a pathogenic FUS variant.
  • The study highlights phenotypic variability linked to mutations in different regions of the KIF5A gene.