Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Multicenter Study
. 2008 Aug 15;23(11):1596-601.
doi: 10.1002/mds.22186.

Huntington CAG repeat size does not modify onset age in familial Parkinson's disease: the GenePD study

Affiliations
Multicenter Study

Huntington CAG repeat size does not modify onset age in familial Parkinson's disease: the GenePD study

Christopher F McNicoll et al. Mov Disord. .

Abstract

The ATP/ADP ratio reflects mitochondrial function and has been reported to be influenced by the size of the Huntington disease gene (HD) repeat. Impaired mitochondrial function has long been implicated in the pathogenesis of Parkinson's disease (PD), and therefore, we evaluated the relationship of the HD CAG repeat size to PD onset age in a large sample of familial PD cases. PD affected siblings (n = 495), with known onset ages from 248 families, were genotyped for the HD CAG repeat. Genotyping failed in 11 cases leaving 484 for analysis, including 35 LRRK2 carriers. All cases had HD CAG repeats (range, 15-34) below the clinical range for HD, although 5.2% of the sample (n = 25) had repeats in the intermediate range (the intermediate range lower limit = 27; upper limit = 35 repeats), suggesting that the prevalence of intermediate allele carriers in the general population is significant. No relation between the HD CAG repeat size and the age at onset for PD was found in this sample of familial PD.

PubMed Disclaimer

Figures

Figure 1
Figure 1
The box plot shows the median and 25th and 75th percentiles with whiskers extending to the 5th and 95th percentiles of the onset age of PD for each CAG repeat length.

Comment in

References

    1. de Lau LM, Giesbergen PC, de Rijk MC, Hofman A, Koudstaal PJ, Breteler MM. Incidence of parkinsonism and Parkinson disease in a general population: the Rotterdam Study. Neurology. 2004;63(7):1240–4. - PubMed
    1. Morgante L, Rocca WA, Di Rosa AE, De Domenico P, Grigoletto F, Meneghini F, Reggio A, Savettieri G, Castiglione MG, Patti F, et al. Prevalence of Parkinson’s disease and other types of parkinsonism: a door-to-door survey in three Sicilian municipalities. The Sicilian Neuro-Epidemiologic Study (SNES) Group. Neurology. 1992 Oct;42(10):1901–7. - PubMed
    1. Klein C, Schlossmacher MG. The genetics of Parkinson disease: implications for neurological care. Nature Clinical Practice Neurology. 2006;2(3):136–46. - PubMed
    1. Polymeropoulos MH, Lavedan C, Leroy E, Ide SE, Dehejia A, Dutra A, Pike B, Root H, Rubenstein J, Boyer R, Stenroos ES, Chandrasekharappa S, Athanassiadou A, Papapetropoulos T, Johnson WG, Lazzarini AM, Duvoisin RC, Di Iorio G, Golbe LI, Nussbaum RL. Mutation in the alpha-synuclein gene identified in families with Parkinson’s disease. Science. 1997;276(5321):2045–7. - PubMed
    1. Kitada T, Asakawa S, Hattori N, Matsumine H, Yamamura Y, Minoshima S, Yokochi M, Mizuno Y, Shimizu N. Mutations in the parkin gene cause autosomal recessive juvenile parkinsonism. Nature. 1998;392(6676):605–8. - PubMed

Publication types