Towards early detection: Positive RT-QuICR is associated with slower disease progression
Author: Maitena San Juan Creutzfeldt-Jakob neurodegenerative disease (CJD) is a prion disorder caused by the misfolded form of the cellular prion protein (PrPC), also known as PrPSc. Its most common variant is the sporadic form (sCJD), which accounts for approximately 90% of the cases of prion disease diagnosed in humans and is of [...]
Mood disturbances could be an early sign of Creutzfeldt-Jakob disease
Author: Eva Fernández-Muñoz Creutzfeldt-Jakob disease (CJD) is a rare and rapidly progressive neurodegenerative disorder caused by the misfolding of the cellular prion protein (PrPC) into a misfolded form called PrPSc, which accumulates in the brain, causing neuronal damage and spongiform degeneration. The sporadic form (sCJD) is the most common form, accounting for approximately [...]
The Spanish Foundation for Prion Diseases submits its application to the Ministry of Health for the inclusion of prion diseases in the ELA Act.
Author: Joaquín Castilla An essential step in the defence of the rights of patients and their families In an effort to guarantee the recognition and protection of the rights of people affected by prion diseases, the Spanish Foundation for Prion Diseases has exercised its right to participate in the Prior Public Consultation [...]
New strategies to understand and combat sporadic Creutzfeldt-Jakob disease through integration of genetic data
Author: Nuno Anjo Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare, fatal and rapidly progressive neurodegenerative disease for which there are still no effective treatments. It is characterised by the misfolding of the normal prion protein (PrPC) into an abnormal form called PrPSc or Prion, which accumulates in the brain, causing neuronal damage [...].
ION717 clinical trial completes patient enrolment
Author: Eric Minikel (translated by Joaquín Castilla) Ionis Pharmaceuticals has announced (see original letter) this morning that it has completed enrolment of PrProfile, its Phase 1/2a clinical trial of ION717, an ASO drug designed to reduce PrP levels in prion diseases. The study is now listed as "Active, without [...]
Scientists discover early brain alterations in people at risk of developing hereditary Creutzfeldt-Jakob disease
Author: Cristina Sampedro Prion diseases can occur spontaneously or genetically, the latter being caused by a mutation in the prion protein (PrP), which are rare but devastating neurodegenerative disorders that affect both humans and animals. The main types of genetic prion diseases are genetic Creutzfeldt-Jakob disease (gCJD), genetic Creutzfeldt-Jakob disease (gCJD), genetic Creutzfeldt-Jakob disease (gCJD), genetic [...]
Amyotrophic Lateral Sclerosis joins the group of diseases known as prion-like diseases.
Author: Jorge Moreno Charco Prion diseases, or Transmissible Spongiform Encephalopathies (TSE), could be defined as a group of neurodegenerative diseases related to the misfolding and propagation of the endogenous prion protein through the central nervous system. This misfolded protein, in addition to spreading uncontrollably through the patient's brain, is highly neurotoxic, which can lead to a [...]
Research priorities on the role of α-synuclein in the pathogenesis of Parkinson's Disease
Authors: Nerea Larrañaga and Paula Marco Scientists from the International Parkinson and Movement Disorders Society (MDS) have published a paper highlighting the main research priorities for α-synuclein (αSyn) protein research in Parkinson's disease. This review, led by an international team of experts, focuses on addressing key aspects of α-synuclein [...]
Global Update on Prion Diseases: What does the latest Epidemiological Atlas tell us?
Authors: Paula Marco and Nerea Larrañaga Human prion diseases (such as Creutzfeldt-Jakob disease (CJD), fatal familial insomnia (FFI) and Gerstmann-Sträussler-Scheinker syndrome (GSS), among others) are a group of rapidly progressive, fatal and potentially transmissible neurodegenerative disorders caused by the misfolding of the physiological protein PrPC, which results in the misfolding of the protein PrPC.
Expansion and reactivation of Ionis PrProfile (ION717) clinical trial for people with symptomatic prion disease
Author: Joaquín Castilla Ionis Pharmaceuticals has just announced that the recruitment pause for its PrProfile (ION717) study for people with symptomatic prion disease has been lifted. Important information to note: Resumption of recruitment: After a planned pause, recruitment for the PrProfile clinical trial has resumed as of [...]
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