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Mutations in VPS13D lead to a new recessive ataxia with spasticity and mitochondrial defects.

Eunju SeongRyan InsoleraMarija DulovicErik-Jan KamsteegJoanne TrinhNorbert BrüggemannErin SandfordSheng LiAyse Bilge OzelJun Z LiTamison JewettAnneke J A KievitAlexander MünchauVikram ShakkottaiChristine KleinCatherine A CollinsKatja LohmannBart P van de WarrenburgMargit Burmeister
Published in: Annals of neurology (2018)
Our study demonstrates that compound heterozygous mutations in VPS13D cause movement disorders along the ataxia-spasticity spectrum, making VPS13D the fourth VPS13 paralog involved in neurological disorders. Ann Neurol 2018.
Keyphrases
  • early onset
  • spinal cord injury
  • botulinum toxin
  • upper limb
  • oxidative stress
  • neural network