Article
The role of protein denaturation energetics and molecular chaperones in the aggregation and mistargeting of mutants causing primary hyperoxaluria type I.
PloS one - 1 Jan 2013
Mesa-Torres Noel, Fabelo-Rosa Israel, Riverol Debora, Yunta Cristina, Albert Armando, Salido Eduardo, Pey Angel L
Abstract excerpt
Primary hyperoxaluria type I (PH1) is a conformational disease which result in the loss of alanine:glyoxylate aminotransferase (AGT) function. The study of AGT has important implications for protein folding and trafficking because PH1 mutants may cause protein aggregation and mitochondrial mistargeting. We herein describe a multidisciplinary study aimed to understand the molecular basis of protein aggregation and...
Topics
- Animals
- CHO Cells
- Cricetulus
- HSC70 Heat-Shock Proteins
- Humans
- Hyperoxaluria, Primary
- Models, Molecular
- Mutation
- Protein Conformation
- Protein Denaturation