The brain’s powerhouses are damaged in Creutzfeldt-Jacob disease
Mitochondrial failure contributes to Creutzfeldt-Jacob disease, study suggests.
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A study performed by researchers from the University of Bergen, Norway, and the University of Vienna, Austria, shows damage of the mitochondria –the cell’s microscopic powerhouses – in the brain of people with Creutzfeldt-Jacob disease.
The researchers found that the mitochondrial power generator (known as the respiratory chain) is severely impaired in brain cells from people who died with Creutzfeldt-Jacob disease.
“These mitochondrial defects were widespread in the brain and correlated with the severity of disease,” says Professor Charalampos Tzoulis at the Department of Clinical Medicine, University of Bergen.
The study is published in Acta Neuropathologica Communications.
Suggests contribution to disease
Damaged mitochondria will no longer be able to provide the energy required for neuronal maintenance and function.
“These findings strongly suggest that mitochondrial failure contributes to the pathogenesis of Creutzfeldt-Jacob disease,” says Tzoulis.
Hoping for new treatment
Tzoulis highlight the need to better understand how mitochondrial failure contributes to Creutzfeldt-Jacob disease.
“We hope that this study may be exploited towards developing treatments for this incurable and deadly condition,” says Charalampos Tzoulis.
Facts
Creutzfeldt-Jacob disease
- Creutzfeldt-Jacob disease is a rare and rapid progressive and severe disease that cause loss of brain tissue.
- It is a lethal brain disease that annually affects 1-2 people per million population, and causes death within 4-24 months.
- The disease normally affects individuals above the age of 60, and causes a combination of invaliding symptoms, often dementia and movement disorders.
- In spite of years of research, there is currently no cure.
