Myoclonic dystonia phenotype related to a novel calmodulin-binding transcription activator 1 sequence variant

Neurogenetics. 2021 May;22(2):137-141. doi: 10.1007/s10048-021-00637-6. Epub 2021 Mar 6.

Abstract

Intragenic rearrangements and sequence variants in the calmodulin-binding transcription activator 1 gene (CAMTA1) can result in a spectrum of clinical presentations, most notably congenital ataxia with or without intellectual disability. We describe for the first time a myoclonic dystonia-predominant phenotype associated with a novel CAMTA1 sequence variant. Furthermore, by identifying an additional, recurrent CAMTA1 sequence variant in an individual with a more typical neurodevelopmental disease manifestation, we contribute to the elucidation of phenotypic variability associated with CAMTA1 gene mutations.

Keywords: CAMTA1; Dystonia; Myoclonus; Neurodevelopmental disorder.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Calcium-Binding Proteins / genetics*
  • Child, Preschool
  • Codon, Nonsense*
  • Dystonic Disorders / genetics*
  • Exome Sequencing
  • Female
  • Frameshift Mutation*
  • Genetic Association Studies
  • Hearing Loss / genetics
  • Humans
  • Intellectual Disability / genetics
  • Male
  • Pedigree
  • Phenotype
  • Sequence Deletion*
  • Trans-Activators / genetics*
  • Vision Disorders / genetics

Substances

  • CAMTA1 protein, human
  • Calcium-Binding Proteins
  • Codon, Nonsense
  • Trans-Activators

Supplementary concepts

  • Myoclonic dystonia