Display Settings:

Format

Send to:

Choose Destination
See comment in PubMed Commons below
Science. 1997 Sep 26;277(5334):1990-3.

Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain.

Author information

  • 1Department of Neurology, Massachusetts General Hospital, Boston, MA 02114, USA.

Abstract

The cause of neurodegeneration in Huntington's disease (HD) is unknown. Patients with HD have an expanded NH2-terminal polyglutamine region in huntingtin. An NH2-terminal fragment of mutant huntingtin was localized to neuronal intranuclear inclusions (NIIs) and dystrophic neurites (DNs) in the HD cortex and striatum, which are affected in HD, and polyglutamine length influenced the extent of huntingtin accumulation in these structures. Ubiquitin was also found in NIIs and DNs, which suggests that abnormal huntingtin is targeted for proteolysis but is resistant to removal. The aggregation of mutant huntingtin may be part of the pathogenic mechanism in HD.

PMID:
9302293
[PubMed - indexed for MEDLINE]
Free full text
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Icon for HighWire
    Loading ...
    Write to the Help Desk