Skip to main content

Main menu

  • Home
  • Content
    • Latest
    • Ahead of print
    • Archive
  • Info for
    • Authors
    • Reviewers
    • Subscribers
    • Institutions
    • Advertisers
  • About Us
    • About Us
    • Editorial Office
    • Editorial Board
  • More
    • Alerts
    • Feedback
    • Folders
    • Help
  • Other Publications
    • Saudi Medical Journal

User menu

  • My alerts
  • Log in

Search

  • Advanced search
Neurosciences Journal
  • Other Publications
    • Saudi Medical Journal
  • My alerts
  • Log in
Neurosciences Journal

Advanced Search

  • Home
  • Content
    • Latest
    • Ahead of print
    • Archive
  • Info for
    • Authors
    • Reviewers
    • Subscribers
    • Institutions
    • Advertisers
  • About Us
    • About Us
    • Editorial Office
    • Editorial Board
  • More
    • Alerts
    • Feedback
    • Folders
    • Help
  • Follow psmmc on Twitter
  • Visit psmmc on Facebook
  • RSS
Case ReportCASE REPORTS
Open Access

A survey of (CAG)n repeats causing juvenile Huntington disease in an Iranian family with 4 affected members

Mehrdokht Mazdeh, Hamid Pour-Jafari, Ali Ghaleiha and Bita Pour-Jafari
Neurosciences Journal July 2009, 14 (3) 273-276;
Mehrdokht Mazdeh
Department of Neurology, School of Medicine, Hamadan University of Medical Sciences, Hamadan, Iran.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Hamid Pour-Jafari
Department of Neurology, School of Medicine, Hamadan University of Medical Sciences, Hamadan, Iran.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Ali Ghaleiha
Department of Neurology, School of Medicine, Hamadan University of Medical Sciences, Hamadan, Iran.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Bita Pour-Jafari
Department of Neurology, School of Medicine, Hamadan University of Medical Sciences, Hamadan, Iran.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • eLetters
  • Info & Metrics
  • PDF
Loading

Abstract

Huntington’s disease is caused by a trinucleotide repeat expansion (CAG)n in the gene coding for Huntingtin (Htt) and is one of the several polyglutamine diseases. Its physical symptoms occur in a large range of ages, with a mean occurrence in a person’s late 40’s and early 50’s. Almost all references indicated that if the age of onset is below 20 years then it is known as juvenile HD. Our case was an Iranian family with 4 affected siblings (2 sisters and 2 brothers). In addition to 4 affected children, they had 5 normal male progenies. There was no any other case in their family history. The onset age of the disease in our case family was 20 to 25 years. Their parents were unaffected and nonconsanguineous. Analysis of the pathogenic (CAG)n repeat region of the HD gene for the affected members have showed an expansion allele with 46, 50, 46, and 44 repeats in 4 affected siblings. Our results indicated that the age of 20 years maybe is not a stable limit point for all cases of juvenile HD, and perhaps onset ages are related with the CAG repeat sizes in such individuals.

  • Copyright: © Neurosciences

Neurosciences is an Open Access journal and articles published are distributed under the terms of the Creative Commons Attribution-NonCommercial License (CC BY-NC). Readers may copy, distribute, and display the work for non-commercial purposes with the proper citation of the original work.

PreviousNext
Back to top

In this issue

Neurosciences Journal: 14 (3)
Neurosciences Journal
Vol. 14, Issue 3
1 Jul 2009
  • Table of Contents
  • Cover (PDF)
  • Index by author
Download PDF
Email Article

Thank you for your interest in spreading the word on Neurosciences Journal.

NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

Enter multiple addresses on separate lines or separate them with commas.
A survey of (CAG)n repeats causing juvenile Huntington disease in an Iranian family with 4 affected members
(Your Name) has sent you a message from Neurosciences Journal
(Your Name) thought you would like to see the Neurosciences Journal web site.
Citation Tools
A survey of (CAG)n repeats causing juvenile Huntington disease in an Iranian family with 4 affected members
Mehrdokht Mazdeh, Hamid Pour-Jafari, Ali Ghaleiha, Bita Pour-Jafari
Neurosciences Journal Jul 2009, 14 (3) 273-276;

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
Share
A survey of (CAG)n repeats causing juvenile Huntington disease in an Iranian family with 4 affected members
Mehrdokht Mazdeh, Hamid Pour-Jafari, Ali Ghaleiha, Bita Pour-Jafari
Neurosciences Journal Jul 2009, 14 (3) 273-276;
Twitter logo Facebook logo Mendeley logo
  • Tweet Widget
  • Facebook Like
  • Google Plus One
Bookmark this article

Jump to section

  • Article
  • eLetters
  • Info & Metrics
  • PDF

Related Articles

  • No related articles found.
  • Google Scholar

Cited By...

  • No citing articles found.
  • Google Scholar

More in this TOC Section

  • A novel variant of RBCK1 gene causes mild polyglucosan myopathy
  • Unusual presentation of neurobrucellosis in Jeddah, Kingdom of Saudi Arabia
  • Sjogren’s syndrome associated with bilateral peripheral facial paralysis
Show more CASE REPORTS

Similar Articles

Navigate

  • home

More Information

  • Help

Additional journals

  • All Topics

Other Services

  • About

© 2025 Neurosciences Journal Neurosciences is copyright under the Berne Convention and the International Copyright Convention. All rights reserved. Neurosciences is an Open Access journal and articles published are distributed under the terms of the Creative Commons Attribution-NonCommercial License (CC BY-NC). Readers may copy, distribute, and display the work for non-commercial purposes with the proper citation of the original work. Electronic ISSN 1658-3183. Print ISSN 1319-6138.

Powered by HighWire