Article
Maturation of wild-type and mutated frataxin by the mitochondrial processing peptidase.
Human molecular genetics - 1 Sept 1998
Koutnikova H, Campuzano V, Koenig M
Abstract excerpt
Frataxin is a mitochondrial protein deficient in Friedreich ataxia (FRDA) and which is associated with abnormal intramitochondrial iron handling. We identified the mitochondrial processing peptidase beta (MPPbeta) as a frataxin protein partner using the yeast two-hybrid assay. In in vitro assays,...
Topics
- Animals
- Base Sequence
- COS Cells
- DNA Primers
- Dimerization
- Friedreich Ataxia
- Gene Expression
- Humans
- In Vitro Techniques
- Iron-Binding Proteins
- Metalloendopeptidases
- Mitochondria
- Mutation
- Phosphotransferases (Alcohol Group Acceptor)
- Polymerase Chain Reaction
- Protein Processing, Post-Translational
- Recombinant Proteins
- Saccharomyces cerevisiae