Comprehensive screening of a North American Parkinson's disease cohort for LRRK2 mutation

Neurodegener Dis. 2007;4(5):386-91. doi: 10.1159/000105160. Epub 2007 Jul 6.

Abstract

Background: Recently, mutations in LRRK2 encoding the protein dardarin have been linked to an autosomal dominant form of parkinsonism.

Objective: To identify mutations causing Parkinson's disease (PD) in a cohort of North Americans with familial PD.

Methods: We sequenced exons 1-51 of LRRK2 in 79 unrelated North American PD patients reporting a family history of the disease.

Results: One patient had a missense mutation (Thr2356Ile) while two others had the common Gly2019Ser mutation. In addition, 1 patient had a 4-bp deletion in close proximity to the exon 19 splice donor (IVS20+4delGTAA) that in vitro abrogates normal splicing.

Conclusions: Our observations in the 79 North American patients indicate that mutations in LRRK2 are associated with approximately 5% of PD cases with a positive family history. The results also show that G2019S represents approximately half of the LRRK2 mutations in United States PD cases with a family history of the disease. We have identified two novel mutations in LRRK2.

Publication types

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

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Alternative Splicing / genetics
  • Amino Acid Sequence
  • Amino Acid Substitution / genetics
  • Base Sequence
  • Brain Chemistry / genetics*
  • Cohort Studies
  • DNA Mutational Analysis
  • Genetic Markers / genetics
  • Genetic Predisposition to Disease / genetics*
  • Genetic Testing
  • Humans
  • Leucine-Rich Repeat Serine-Threonine Protein Kinase-2
  • Male
  • Middle Aged
  • Molecular Sequence Data
  • Mutation / genetics*
  • Mutation, Missense / genetics
  • North America
  • Parkinson Disease / genetics*
  • Parkinson Disease / metabolism
  • Parkinson Disease / physiopathology
  • Protein Serine-Threonine Kinases / genetics*
  • RNA Splice Sites / genetics

Substances

  • Genetic Markers
  • RNA Splice Sites
  • LRRK2 protein, human
  • Leucine-Rich Repeat Serine-Threonine Protein Kinase-2
  • Protein Serine-Threonine Kinases