Glucocerebrosidase mutations in diffuse Lewy body disease

Parkinsonism Relat Disord. 2011 Jan;17(1):55-7. doi: 10.1016/j.parkreldis.2010.09.009.

Abstract

Clinicogenetic and pathological studies have shown that mutations of the glucocerebrosidase gene (GBA) are a risk factor for Parkinson's disease and Lewy body disorders. In the present study, we have identified GBA mutations in 6.8% (4/59) of cases with a pathological diagnosis of diffuse Lewy body disease. Taken with previous studies, it appears that GBA mutations are associated with a more diffuse pattern of Lewy body distribution involving the cerebral cortex than the brainstem/limbic distribution observed in typical Parkinson's disease.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Brain / enzymology
  • Brain / pathology
  • Brain Stem / enzymology
  • Brain Stem / pathology
  • Cerebral Cortex / enzymology
  • Cerebral Cortex / pathology
  • DNA / genetics
  • Female
  • Gaucher Disease / enzymology
  • Gaucher Disease / genetics
  • Glucosylceramidase / genetics*
  • Humans
  • Lewy Body Disease / enzymology*
  • Lewy Body Disease / genetics*
  • Lewy Body Disease / pathology
  • Limbic System / enzymology
  • Limbic System / pathology
  • Male
  • Mutation / genetics

Substances

  • DNA
  • Glucosylceramidase