Genetic analysis of synphilin-1 in familial Parkinson's disease

Neurobiol Dis. 2001 Apr;8(2):317-23. doi: 10.1006/nbdi.2000.0326.

Abstract

alpha-Synuclein is present in Lewy bodies of patients with both sporadic and familial Parkinson's disease. However, pathogenic mutations Ala30Pro and Ala53Thr in alpha-synuclein are rare causes of disease. Synphilin-1 has been demonstrated to associate with alpha-synuclein and promote the formation of cytosolic inclusions in vitro. Two-point genetic linkage analysis of a dinucleotide repeat within the synphilin-1 gene initially implicated this locus as a cause of Parkinson's disease in three of nine families. However, subsequent haplotype, sequencing, and association analyses in these three families and an independent case-control series suggest that variability within the locus does not confer susceptibility to Parkinson's disease.

Publication types

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

MeSH terms

  • Aged
  • Alleles
  • Carrier Proteins / genetics*
  • Carrier Proteins / metabolism
  • Chromosome Mapping
  • DNA Mutational Analysis
  • Female
  • Genetic Linkage / genetics*
  • Haplotypes
  • Humans
  • Male
  • Middle Aged
  • Nerve Tissue Proteins / genetics*
  • Nerve Tissue Proteins / metabolism
  • Parkinsonian Disorders / genetics*
  • Parkinsonian Disorders / metabolism
  • Parkinsonian Disorders / physiopathology
  • Pedigree

Substances

  • Carrier Proteins
  • Nerve Tissue Proteins
  • SNCAIP protein, human