Very early onset Huntington's disease: genetic mechanism and risk to siblings
Language: 
English
Short Title: 
Very early onset Huntington's disease
Abstract: 

A study of very early onset Huntington's disease (VEOHD) has shown that at least 38% of gene-carrying sibs also develop symptoms before the age of 10, thus improving the genetic risk for those sibs who remain healthy. The prevalence of VEOHD among sibs shows that mutation during spermatogenesis is most unlikely to account for these uncommon cases. The data suggest that two mechanisms contribute to VEOHD: modification by many genes (individually of small effect), and an epigenetic mechanism occurring when transmission is through a series of males.

Author(s): 
Clarke, D. J.
Bundey, S.
Item Type: 
Journal Article
Publication Title: 
Clinical Genetics
Journal Abbreviation: 
Clin. Genet.
Publication Date: 
1990-09
Publication Year: 
1990
Pages: 
180-186
Volume: 
38
Issue: 
3
ISSN: 
0009-9163
Library Catalog: 
PubMed
Extra: 
PMID: 2146048

Turabian/Chicago Citation

D. J. Clarke and S. Bundey. 1990-09. "Very early onset Huntington's disease: genetic mechanism and risk to siblings." Clinical Genetics 38: 3: 180-186.

Wikipedia Citation

<ref> {{Cite journal | doi = | issn = 0009-9163 | volume = 38 | pages = 180-186 | last = Clarke | first = D. J. | coauthors = Bundey, S. | title = Very early onset Huntington's disease: genetic mechanism and risk to siblings | journal = Clinical Genetics | date = 1990-09 | pmid = | pmc = }} </ref>