Clinical, 18F-dopa PET, and genetic analysis of an ethnic Chinese kindred with early-onset parkinsonism and parkin gene mutations

Mov Disord. 2002 Jul;17(4):670-5. doi: 10.1002/mds.10184.

Abstract

We report on clinical (18)F-labeled 6-fluorodopa ((18)F-dopa) positron emission tomography (PET) and molecular genetic analyses of an ethnic Chinese family in which three siblings presented with early-onset Parkinson's disease. As described in some parkin patients, neither sleep benefit nor diurnal fluctuation was noted. Interestingly, depression, anxiety, and obsessive-compulsive disorders were manifest. The (18)F-dopa PET scans showed bilateral presynaptic dopaminergic dysfunction without marked lateralization. Molecular genetic analysis showed identical chromosome 6 haplotypes inherited by affected subjects, with alternate allelic deletions of parkin exons 3 and 4. Furthermore, mRNA analyses identified aberrantly spliced parkin transcripts, suggesting that unusual parkin protein isoforms may be expressed in the brain and retain some function.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adult
  • Alleles
  • Asian People / genetics*
  • Caudate Nucleus / diagnostic imaging
  • Caudate Nucleus / physiopathology
  • Chromosome Deletion
  • Chromosomes, Human, Pair 6
  • Dominance, Cerebral / physiology
  • Dopamine / physiology
  • Female
  • Fluorodeoxyglucose F18*
  • Haplotypes
  • Humans
  • Ligases / genetics*
  • Male
  • Mutation / genetics*
  • Parkinson Disease / diagnostic imaging
  • Parkinson Disease / genetics*
  • Parkinson Disease / physiopathology
  • Pedigree
  • Putamen / diagnostic imaging
  • Putamen / physiopathology
  • RNA, Messenger / genetics
  • Receptors, Presynaptic / physiology
  • Taiwan
  • Tomography, Emission-Computed*
  • Ubiquitin-Protein Ligases*

Substances

  • RNA, Messenger
  • Receptors, Presynaptic
  • Fluorodeoxyglucose F18
  • Ubiquitin-Protein Ligases
  • parkin protein
  • Ligases
  • Dopamine