Article
Molecular chaperone disorders: defective Hsp60 in neurodegeneration.
Current topics in medicinal chemistry - 1 Jan 2012
Bross Peter, Magnoni Raffaella, Bie Anne Sigaard
Abstract excerpt
Chaperonins, a subgroup of molecular chaperones, form ring-shaped structures and assist folding of proteins by enclosing them in their inner cavity. The mitochondrial Hsp60/Hsp10 chaperonin system is essential for cell viability and only a very small number of mutations causing human disease have so far been found that appear to selectively affect neuronal tissues. We here review the knowledge on the mammalian...
Topics
- Animals
- Chaperonin 10
- Chaperonin 60
- Gene Expression Regulation
- Humans
- Mammals
- Mice
- Mice, Knockout
- Mitochondria
- Mitochondrial Proteins
- Molecular Chaperones
- Mutation
- Neurodegenerative Diseases
- Neuroglia
- Protein Folding
- Spastic Paraplegia, Hereditary