Journal article
The Association between CAG Repeat Length and Age of Onset of Juvenile-Onset Huntington’s Disease
Brain sciences, Vol.10(9), p.575
09/01/2020
DOI: 10.3390/brainsci10090575
PMID: 32825467
Abstract
There is a known negative association between cytosine–adenine–guanine (CAG) repeat length and the age of motor onset (AMO) in adult-onset Huntington’s Disease (AOHD). This relationship is less clear in patients with juvenile-onset Huntington’s disease (JOHD), however, given the rarity of this patient population. The aim of this study was to investigate this relationship amongst a relatively large group of patients with JOHD using data from the Kids-JOHD study. Additionally, we analyzed data from the Enroll-HD platform and the Predict-HD study to compare the relationship between CAG repeat length and AMO amongst patients with AOHD to that amongst patients with JOHD using linear regression models. In line with previous reports, the variance in AMO that was predicted by CAG repeat length was 59% (
p
< 0.0001) in the Predict-HD study and 57% from the Enroll-HD platform (
p
< 0.0001). However, CAG repeat length predicted 84% of the variance in AMO amongst participants from the Kids-JOHD study (
p
< 0.0001). These results indicate that there may be a stronger relationship between CAG repeat length and AMO in patients with JOHD as compared to patients with AOHD. These results provide additional information that may help to model disease progression of JOHD, which is beneficial for the planning and implementation of future clinical trials.
Details
- Title: Subtitle
- The Association between CAG Repeat Length and Age of Onset of Juvenile-Onset Huntington’s Disease
- Creators
- Jordan L Schultz - Department of Psychiatry, Carver College of Medicine at the University of Iowa, Iowa City, IA 52242, USAAmelia D Moser - Department of Psychology and Neuroscience, University of Colorado Boulder, Boulder, CO 80309, USAPeg C Nopoulos - Department of Psychiatry, Carver College of Medicine at the University of Iowa, Iowa City, IA 52242, USA
- Resource Type
- Journal article
- Publication Details
- Brain sciences, Vol.10(9), p.575
- DOI
- 10.3390/brainsci10090575
- PMID
- 32825467
- NLM abbreviation
- Brain Sci
- ISSN
- 2076-3425
- eISSN
- 2076-3425
- Publisher
- MDPI
- Grant note
- DOI: 10.13039/100000065, name: National Institute of Neurological Disorders and Stroke, award: R01NS055903
- Language
- English
- Date published
- 09/01/2020
- Academic Unit
- Neurology; Psychiatry; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Pharmacy Practice and Science
- Record Identifier
- 9984070775202771
Metrics
29 Record Views