A mutation of the Wilson disease protein, ATP7B, is degraded in the proteasomes and forms protein aggregates. [electronic resource]
Producer: 20010419Description: 967-74 p. digitalISSN:- 0016-5085
- Acetylcysteine -- analogs & derivatives
- Adenosine Triphosphatases -- genetics
- Carrier Proteins -- genetics
- Cation Transport Proteins
- Cell Line
- Copper-Transporting ATPases
- Cysteine Endopeptidases -- drug effects
- Cytoskeleton -- ultrastructure
- Fluorescent Antibody Technique
- Humans
- Leupeptins -- pharmacology
- Microscopy, Confocal
- Microscopy, Electron
- Multienzyme Complexes -- drug effects
- Mutation -- physiology
- Proteasome Endopeptidase Complex
- Tissue Distribution
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Publication Type: Journal Article; Research Support, Non-U.S. Gov't
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