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PMID: 19006224 Published · ppublish English Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't

Genotype-phenotype correlates in Taiwanese patients with early-onset recessive Parkinsonism.

Movement disorders : official journal of the Movement Disorder Society ·Vol. 24 ·No. 1 ·2009-01-15 ·Pages 104-8

Lee MJ, Mata IF, Lin CH, Tzen KY, Lincoln SJ, Bounds R, Lockhart PJ, Hulihan MM, Farrer MJ, Wu RM

Abstract

We screened for mutations in the PARKIN, DJ-1, and PINK1 genes in a Taiwanese cohort (68 probands; 58 sporadic and 10 familial) with early-onset parkinsonism (EOP, onset <50 years of age). We identified 9 patients harboring mutations in PARKIN (three compound heterozygous and six single heterozygous carriers), 3 patients with heterozygous PINK1 mutations (including two novel substitutions M341I and P209A), and no DJ-1 mutations. Our frequencies of PARKIN (two allele mutation, 4.4%; single allele, 8.8%) and PINK1 (single heterozygous, 4.4%) mutations in Taiwanese-Chinese are similar to those in Caucasian and other Asian EOP patients. Although the role of heterozygosity of recessive genes in EOP remains to be resolved, molecular analysis and functional imaging will play a decisive role in differential diagnosis and determined therapeutic strategy.

MeSH Terms
Adolescent Adult Age of Onset Amino Acid Substitution Asians/genetics China/ethnology Cohort Studies Exons/genetics Female Gene Frequency Genes, Recessive Genotype Humans Intracellular Signaling Peptides and Proteins/genetics Male Middle Aged Mutation, Missense Oncogene Proteins/genetics Parkinsonian Disorders/ethnology,genetics Phenotype Polymerase Chain Reaction Protein Deglycase DJ-1 Protein Kinases/genetics RNA, Messenger/genetics Taiwan/epidemiology Ubiquitin-Protein Ligases/genetics Young Adult
Chemicals
Intracellular Signaling Peptides and Proteins Oncogene Proteins RNA, Messenger Ubiquitin-Protein Ligases parkin protein Protein Kinases PTEN-induced putative kinase PARK7 protein, human Protein Deglycase DJ-1
Authors & Affiliations
10 authors, click to expand affiliations / ORCID
Lee Ming-Jen
Department of Medical Genetics, National Taiwan University Hospital and College of Medicine, National Taiwan University, Taipei, Taiwan.
Mata Ignacio F
Lin Chin-Hsien
Tzen Kai-Yuan
Lincoln Sarah J
Bounds Rebecca
Lockhart Paul J
Hulihan Mary M
Farrer Matthew J
Wu Ruey-Meei
Article Info
Journal
Movement disorders : official journal of the Movement Disorder Society
Abbr.
Mov Disord
ISSN
1531-8257
Published
2009-01-15
Pages
104-8
Language
English
Region
United States
NLM ID
8610688
Subset
IM
Grants
NINDS NIH HHS · P50 NS40256 · United States
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