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Prions are unconventional, infectious agents composed mainly of proteins. Unlike typical infectious agents such as viruses or bacteria, prions lack genetic material like DNA or RNA. The term "prion" is derived from "proteinaceous infectious particle."
Prions are associated with several neurodegenerative diseases in animals and humans, where they can cause abnormal folding of normal cellular proteins. The misfolded proteins then aggregate and accumulate in the brain, leading to damage and neurological dysfunction.
One well-known prion disease in humans is Creutzfeldt-Jakob Disease (CJD), while in animals, examples include bovine spongiform encephalopathy (BSE), commonly known as "mad cow disease," and scrapie in sheep. These diseases are characterized by progressive neurological deterioration.
The unique and concerning aspect of prions is their ability to induce normal proteins to misfold and become infectious. This self-perpetuating process can lead to the spread of the disease within the organism.
Prion diseases are rare but can have severe consequences. Understanding their biology and transmission mechanisms is crucial for developing strategies to prevent and manage these conditions.
Controlling and curing prion diseases pose significant challenges due to the unique nature of prions and their unconventional mode of infection. Prion diseases are currently considered incurable, and treatment options are limited. However, ongoing research aims to understand the mechanisms of prion diseases better and explore potential interventions.
Dr. Josep Baselga, an internationally renowned medical oncologist, died of this degenerative disease on 21 March 2021. His family launched a micro-donation project and gave all the money collected to the Hospital Clínic de Barcelona. The €223,182 raised from over 1,300 donations played a key role in the implementation of a research project led by Dr. Raquel Sánchez-Valle, Head of the Hospital Clínic Neurology Service and the IDIBAPS Alzheimer’s disease and other cognitive disorders research group.
The Hospital Clínic is a referral centre in Catalonia for the diagnosis of prion diseases, including Creutzfeldt-Jakob disease, a neurodegenerative disease for which there is currently no cure. Dr. Sánchez-Valle explains what the research being carried out on this disease at the Hospital Clínic-IDIBAPS consists of: "At the Hospital Clínic we work with Creutzfeldt-Jakob patients to study how the disease evolves, to improve follow-up tests and to find possible therapeutic targets in order to develop treatments."
In parallel with this, the Hospital Clínic and the BBVA Foundation recently launched a call for applications for the BBVA Foundation - Hospital Clínic de Barcelona Joan Rodés – Josep Baselga Advanced Research Contracts in Oncology and Neurodegenerative Diseases, which will allow professionals with a strong clinical and/or translational research profile in these areas to develop three-year projects at the Hospital Clínic de Barcelona and in a foreign centre of reference.
The BBVA Foundation-Hospital Clínic de Barcelona advanced research contracts are aimed at Health Sciences specialists, with research experience, who want to deepen their scientific expertise by developing a specific project in the area of Oncology or Neurodegenerative Diseases.
Josep Baselga studied medicine at the Universitat Autònoma de Barcelona, and began his training in Internal Medicine at the Hospital Universitari Vall d'Hebron. He went on to complete his training in this specialty at the Kings County Hospital in Brooklyn, and then in Oncology at the Memorial Sloan Kettering Cancer Center in New York. He returned to Spain in 1996 as a Full Professor at the Universitat Autònoma de Barcelona, and as a coordinator and Head of the Medical Oncology Service at the Hospital Universitari Vall d'Hebron. During his leadership, he created a multidisciplinary, pioneering Oncology Department, an international benchmark in which patient care was integrated with a basic, clinical and translational research programme, in order to transfer the progress made in the laboratory to patients as quickly as possible.
In 2010 he moved to Boston to head the oncology division – with over 100 researchers – at Massachusetts General Hospital / Harvard Medical School, combining this position with important scientific activity at the Vall d’Hebron Institute of Oncology (VHIO) and the co-management, alongside Doctor Josep Tabernero, of the BBVA Foundation Tumour Biomarkers Research Programme.