A Comparison of Huntington Disease and Huntington Disease-Like 2 Neuropathology
نویسندگان
چکیده
منابع مشابه
A comparison of huntington disease and huntington disease-like 2 neuropathology.
Huntington disease-like 2 (HDL2) is an autosomal dominant disorder characterized by adult-onset, progressive motor abnormalities, psychiatric disturbances, and dementia ending in premature death. Clinically, it most closely resembles Huntington disease (HD), although a subset of affected individuals have parkinsonian features. Here, we systematically compare 5 HDL2 and 5 HD brains with the hypo...
متن کاملA Drosophila model of Huntington disease-like 2 exhibits nuclear toxicity and distinct pathogenic mechanisms from Huntington disease.
Huntington disease-like 2 (HDL2) and Huntington disease (HD) are adult-onset neurodegenerative diseases characterized by movement disorders, psychiatric disturbances and cognitive decline. Brain tissue from HD and HDL2 patients shows degeneration of the striatum and ubiquitinated inclusions immunoreactive for polyglutamine (polyQ) antibodies. Despite these similarities, the diseases result from...
متن کاملDiagnosis of Huntington disease.
BACKGROUND Huntington disease (HD) is a rare, progressive, and fatal autosomal dominant neurodegenerative disorder, typically of adult onset. METHODS We reviewed the literature concerning the molecular diagnosis of HD. RESULTS The discovery of the genetic etiology of HD, a trinucleotide expansion mutation on chromosome 4p, has led to the development of increasingly reliable and valid diagno...
متن کاملMilestones in huntington disease.
There have been extraordinary advances in our knowledge of the underlying gene, the protein it encodes, various models of disease, and potential targets for effective therapies for Huntington disease. Huntington disease research has increased exponentially in the past 25 years, and we now understand many of the molecular mechanisms underlying the disease. Still, more work needs to be done befor...
متن کاملCase 24. Huntington Disease
● Huntington disease (HD) is a progressive neurological disorder that typically has its onset in the early 40s. ● HD is an autosomal dominant disorder. Genetic testing can determine whether a person has inherited HD prior to the onset of symptoms. ● Because of the lack of treatment for HD, the decision of an asymptomatic at-risk individual to undergo genetic testing is a personal one, based on ...
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ژورنال
عنوان ژورنال: Journal of Neuropathology & Experimental Neurology
سال: 2008
ISSN: 0022-3069,1554-6578
DOI: 10.1097/nen.0b013e31816b4aee