Article
A cellular model that recapitulates major pathogenic steps of Huntington's disease.
Human molecular genetics - 1 Sept 1998
Lunkes A, Mandel J L
Abstract excerpt
To gain insight into the pathogenic mechanisms of Huntington's disease (HD), we have developed a stable cellular model, using a neuroblastoma cell line in which the expression of full-length or truncated forms of wild-type and mutant huntingtin can be induced. While the wild-type forms have the e...
Topics
- Apoptosis
- Cell Line
- Cell Nucleus
- Cytoplasm
- Humans
- Huntingtin Protein
- Huntington Disease
- Inclusion Bodies
- Microscopy, Confocal
- Minisatellite Repeats
- Models, Biological
- Mutation
- Nerve Tissue Proteins
- Nuclear Proteins
- Peptide Fragments
- Peptides
- Protein Processing, Post-Translational
- Transfection