DNA haplotype analysis of CAG repeat in Taiwanese Huntington's disease patients

C. K. Wang; Y. R. Wu; W. L. Hwu; C. M. Chen; L. S. Ro; S. T. Chen; K. Gwinn-Hardy; C. C. Yang; R. M. Wu; T. F. Chen; H. C. Wang; M. C. Chao; M. J. Chiu; C. J. Lu; G. J. Lee-Chen

doi:10.1159/000079938

DNA haplotype analysis of CAG repeat in Taiwanese Huntington's disease patients

C. K. Wang, Y. R. Wu, W. L. Hwu, C. M. Chen, L. S. Ro, S. T. Chen, K. Gwinn-Hardy, C. C. Yang, R. M. Wu, T. F. Chen, H. C. Wang, M. C. Chao, M. J. Chiu, C. J. Lu, G. J. Lee-Chen^*

^*Corresponding author for this work

Department of Life Science

Research output: Contribution to journal › Article › peer-review

16 Citations (Scopus)

Abstract

We studied the expanded CAG repeat and adjacent CCG repeat in 53 Huntington's disease (HD) patients and 172 unrelated normal subjects matched to the patients for ethnic origin. The range of the CAG repeat varied from 38 to 109 in the HD patients and from 10 to 29 in the control group. A significant negative correlation was found between the age at onset and the CAG expansion, with no significant influence of the adjacent CCG repeat on the age at onset by multiple regression analysis. Allelic association using CCG repeat and 2 flanking dinucleotide repeat markers within 150 kb of the HD gene revealed linkage disequilibrium for 2 of 3 markers. Haplotype analysis of 24 HD families using these markers identified 3 major haplotypes underlying 87.5% of HD chromosomes. The data suggested frequent haplotypes in the Taiwanese population on which one or more mutational events leading to the disease occurred.

Original language	English
Pages (from-to)	96-100
Number of pages	5
Journal	European Neurology
Volume	52
Issue number	2
DOIs	https://doi.org/10.1159/000079938
Publication status	Published - 2004

Keywords

CAG repeat
CCG repeat
Dinucleotide repeat markers
Genotyping
Haplotype analysis
Huntington's disease

ASJC Scopus subject areas

Neurology
Clinical Neurology

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10.1159/000079938

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@article{e21028f4054048bd9b186066f8d372dc,

title = "DNA haplotype analysis of CAG repeat in Taiwanese Huntington's disease patients",

abstract = "We studied the expanded CAG repeat and adjacent CCG repeat in 53 Huntington's disease (HD) patients and 172 unrelated normal subjects matched to the patients for ethnic origin. The range of the CAG repeat varied from 38 to 109 in the HD patients and from 10 to 29 in the control group. A significant negative correlation was found between the age at onset and the CAG expansion, with no significant influence of the adjacent CCG repeat on the age at onset by multiple regression analysis. Allelic association using CCG repeat and 2 flanking dinucleotide repeat markers within 150 kb of the HD gene revealed linkage disequilibrium for 2 of 3 markers. Haplotype analysis of 24 HD families using these markers identified 3 major haplotypes underlying 87.5% of HD chromosomes. The data suggested frequent haplotypes in the Taiwanese population on which one or more mutational events leading to the disease occurred.",

keywords = "CAG repeat, CCG repeat, Dinucleotide repeat markers, Genotyping, Haplotype analysis, Huntington's disease",

author = "Wang, {C. K.} and Wu, {Y. R.} and Hwu, {W. L.} and Chen, {C. M.} and Ro, {L. S.} and Chen, {S. T.} and K. Gwinn-Hardy and Yang, {C. C.} and Wu, {R. M.} and Chen, {T. F.} and Wang, {H. C.} and Chao, {M. C.} and Chiu, {M. J.} and Lu, {C. J.} and Lee-Chen, {G. J.}",

year = "2004",

doi = "10.1159/000079938",

language = "English",

volume = "52",

pages = "96--100",

journal = "European Neurology",

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TY - JOUR

T1 - DNA haplotype analysis of CAG repeat in Taiwanese Huntington's disease patients

AU - Wang, C. K.

AU - Wu, Y. R.

AU - Hwu, W. L.

AU - Chen, C. M.

AU - Ro, L. S.

AU - Chen, S. T.

AU - Gwinn-Hardy, K.

AU - Yang, C. C.

AU - Wu, R. M.

AU - Chen, T. F.

AU - Wang, H. C.

AU - Chao, M. C.

AU - Chiu, M. J.

AU - Lu, C. J.

AU - Lee-Chen, G. J.

PY - 2004

Y1 - 2004

N2 - We studied the expanded CAG repeat and adjacent CCG repeat in 53 Huntington's disease (HD) patients and 172 unrelated normal subjects matched to the patients for ethnic origin. The range of the CAG repeat varied from 38 to 109 in the HD patients and from 10 to 29 in the control group. A significant negative correlation was found between the age at onset and the CAG expansion, with no significant influence of the adjacent CCG repeat on the age at onset by multiple regression analysis. Allelic association using CCG repeat and 2 flanking dinucleotide repeat markers within 150 kb of the HD gene revealed linkage disequilibrium for 2 of 3 markers. Haplotype analysis of 24 HD families using these markers identified 3 major haplotypes underlying 87.5% of HD chromosomes. The data suggested frequent haplotypes in the Taiwanese population on which one or more mutational events leading to the disease occurred.

AB - We studied the expanded CAG repeat and adjacent CCG repeat in 53 Huntington's disease (HD) patients and 172 unrelated normal subjects matched to the patients for ethnic origin. The range of the CAG repeat varied from 38 to 109 in the HD patients and from 10 to 29 in the control group. A significant negative correlation was found between the age at onset and the CAG expansion, with no significant influence of the adjacent CCG repeat on the age at onset by multiple regression analysis. Allelic association using CCG repeat and 2 flanking dinucleotide repeat markers within 150 kb of the HD gene revealed linkage disequilibrium for 2 of 3 markers. Haplotype analysis of 24 HD families using these markers identified 3 major haplotypes underlying 87.5% of HD chromosomes. The data suggested frequent haplotypes in the Taiwanese population on which one or more mutational events leading to the disease occurred.

KW - CAG repeat

KW - CCG repeat

KW - Dinucleotide repeat markers

KW - Genotyping

KW - Haplotype analysis

KW - Huntington's disease

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SN - 0014-3022

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JO - European Neurology

JF - European Neurology

IS - 2

ER -

DNA haplotype analysis of CAG repeat in Taiwanese Huntington's disease patients

Abstract

Keywords

ASJC Scopus subject areas

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