Multiple system atrophy and C9orf72 hexanucleotide repeat expansions in a cohort of Italian patients

Neurobiol Aging. 2022 Apr:112:12-15. doi: 10.1016/j.neurobiolaging.2021.12.003. Epub 2021 Dec 20.

Abstract

Exanucleotide expansions in C9orf72 gene have been described as potential risk factor in some patients with Multiple system atrophy (MSA) and other forms of atypical parkinsonism. The goal of our study was to extend the knowledge on the involvement of C9orf72 in MSA studying a cohort of 100 patients from Italy. We identified 2 heterozygous patients in the pathological range (> 30 repeats) and 4 heterozygous patients for expansions in the premutation range (20 -30 repeats). Our findings strengthen the previously hypothesized role for this gene as a risk factor for MSA and raise the possibility of a more complex and still unknown involvement of this gene in the heterogeneity of MSA.

Keywords: Atypical parkinsonism; C9orf72; Exanucleotide expansion; Multiple System Atrophy.

MeSH terms

  • Amyotrophic Lateral Sclerosis* / genetics
  • C9orf72 Protein* / genetics
  • Cohort Studies
  • DNA Repeat Expansion / genetics
  • Humans
  • Multiple System Atrophy* / genetics
  • Proteins / genetics

Substances

  • C9orf72 Protein
  • C9orf72 protein, human
  • Proteins