Abstract
Huntington's disease-like 2 (HDL2) is a rare, autosomal dominant, adult-onset, progressive neurodegenerative disorder, genetically, clinically, and pathologically nearly indistinguishable from Huntington's disease (HD). HDL2 is caused by a trinucleotide repeat expansion detectable by genetic testing. Understanding HDL2 may provide clues to the pathogenesis of HD and other neurodegenerative diseases.
Original language | English (US) |
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Title of host publication | Encyclopedia of Movement Disorders |
Publisher | Elsevier Inc. |
Pages | 36-38 |
Number of pages | 3 |
ISBN (Electronic) | 9780123741059 |
ISBN (Print) | 9780123741011 |
DOIs | |
State | Published - Jan 1 2010 |
Keywords
- Atrophy
- Caudate
- Chorea
- Dementia
- Dominant
- Familial
- Hereditary
- Huntington
- Junctophilin
- Neurodegeneration
- Neurotoxicity
- RNA
- Repeat
- Striatum
- Trinucleotide
ASJC Scopus subject areas
- General Medicine
- General Neuroscience