Preparing your results

Our searching services are busy right now. Your search will reload in five seconds.

X
Forgot Password

If you have forgotten your password you can enter your email here and get a temporary password sent to your email.

Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease.

Cell | Mar 31, 2000

http://www.ncbi.nlm.nih.gov/pubmed/10778856

Neurodegenerative disorders like Huntington's disease (HD) are characterized by progressive and putative irreversible clinical and neuropathological symptoms, including neuronal protein aggregates. Conditional transgenic models of neurodegenerative diseases therefore could be a powerful means to explore the relationship between mutant protein expression and progression of the disease. We have created a conditional model of HD by using the tet-regulatable system. Mice expressing a mutated huntingtin fragment demonstrate neuronal inclusions, characteristic neuropathology, and progressive motor dysfunction. Blockade of expression in symptomatic mice leads to a disappearance of inclusions and an amelioration of the behavioral phenotype. We thus demonstrate that a continuous influx of the mutant protein is required to maintain inclusions and symptoms, raising the possibility that HD may be reversible.

Pubmed ID: 10778856 RIS Download

Mesh terms: Animals | Brain | Disease Models, Animal | Female | Gene Expression | Humans | Huntington Disease | Male | Mice | Mice, Inbred C57BL | Mice, Inbred CBA | Mice, Transgenic | Motor Skills Disorders | Mutation | Nerve Tissue Proteins | Nuclear Proteins | Peptides | Phenotype

Research resources used in this publication

Research tools detected in this publication

None found

Data used in this publication

None found

Associated grants

None

Mouse Genome Informatics (Data, Gene Annotation)

Publication data is provided by the National Library of Medicine ® and PubMed ®. Data is retrieved from PubMed ® on a weekly schedule. For terms and conditions see the National Library of Medicine Terms and Conditions.