Tara Hessa, forskningsledare på institutionen för biokemi och biofysik.
Tara Hessa, forskningsledare på institutionen för biokemi och biofysik.
 

Half of all proteins produced within cells are transported from the cytosol to membrane-bound organelles, such as the endoplasmic network. These proteins must be properly flattened to perform their function. When proteins are not properly folded, due to genetic mutations, cell stress or other external factors, they are aggregated into the body. In the long run, it can lead to diseases such as type 2 diabetes or severe neurodegenerative diseases such as dementia.

 

Degradation of mislocalized proteins

The mechanism behind how cells recognize defective secretory and membrane proteins and eliminate the toxic effects of a certain type of error in protein production is unknown. The aim of the research project is therefore to examine how proteins are folded while synthesized on ribosomes and which control mechanisms maintain proper protein growth. We want to investigate this control system by identifying new components and understanding the mechanism behind the machinery that manages the decomposition of localized proteins, says Tara Hessa, researcher at the Department of Biochemistry and Biophysics.

 

May lead to pioneering medicines

According to WHO, there are currently 160,000 people with dementia in Sweden and 47 million worldwide. By examining the basic steps in and the mechanism behind how the cell recognizes how malformed proteins are formed and destroyed, we will improve our knowledge of how we can treat these diseases in the future. The vision is that the pharmaceutical industry eventually gains research results and works on them with the aim of developing new pioneering drugs that can help prevent dementia and other neurodegenerative diseases at an early stage.

"So far, we have studied two model proteins that cause different forms of dementia. In parallel, we study how this type of mutation affects individuals' insulin levels based on how the cell handles and destroys the malignant proteins. We also strive to determine the structure of the protein BAG6, a protein that helps eliminate erroneous proteins, says Tara Hessa.

 

SU – Proteinkvalitetskontroll
Forskningsprojektet Protein Kvalitetskontroll beviljades medel från SSF 2014 och kom igång ordentligt året därpå. Finansieringen från SSF har varit helt avgörande för att forskningsprojektet ska kunna utvecklas.
www.su.se

Publicerad: 26 juni, 2018