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Overview

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    Phenylketonuria (PKU) and Conformational diseases

    PKU is a genetic disease caused by mutations in human phenylalanine hydroxylase (PAH). Our group has contributed to the study of PKU mutations and their effect on the structure, function and stability of PAH, revealing that the decrease in protein stability and increased in vivo turnover is the main molecular pathogenic mechanism in PKU.

  • The interaction of bovine a-lactalbumin with negatively charged membranes (by NMR).

    Interactions with Membranes

    Biological membranes enclose both the cell and its different compartments. An essential feature of a biomembrane is that it is a selectively permeable structure. This means that the cell membrane essentially controls which molecules are allowed to interact with intracellular macromolecules. The vital role of cell membranes has become even more evident as we have realized that more than half of all proteins interact with membranes (either transiently or permanently). Even so, less than 1% of the more than 50 000 experimentally solved protein structures in the protein data bank are of membrane proteins.

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    Molecular Machines

    Molecular machines converts chemical energy into mechanical work. The chaperone GroEL-GroES acts as a folding machine for a wide range essential proteins.