Hereditary Human Prion Diseases: an Update

Prion diseases in humans: an update.

The year 2006 marks 20 years from the first identified bovine spongiform encephalitis in cows and 10 years from the first description of variant Creutzfeldt-Jakob disease in humans. The threatened epidemic in humans now appears unlikely, but psychiatrists need to be aware of recent developments in prion diseases.

Mesenchymal stem cells in osteoarticular diseases: an update

Multipotent mesenchymal stromal cells or mesenchymal stem cells (MSCs) are mainly isolated from bone marrow or fat tissue. Because of their potential of multilineage differentiation towards bone, cartilage and fat tissue, they were initially evaluated to develop innovative strategies for tissue engineering applications. More recently, they have gained interest based on their immunomodulatory pr...

Therapies for human prion diseases.

The pathological foundation of human prion diseases is a result of the conversion of the physiological form of prion protein (PrP(c)) to the pathological protease resistance form PrP(res). Most patients with prion disease have unknown reasons for this conversion and the subsequent development of a devastating neurodegenerative disorder. The conversion of PrP(c) to PrP(res), with resultant propa...

Unveiling the mysteries of hereditary prion diseases in Brazil.

Benign Hereditary Chorea: An Update

Benign hereditary chorea (BHC) is a childhood-onset, hyperkinetic movement disorder normally with little progression of motor symptoms into adult life. The disorder is caused by mutations to the NKX2.1 (TITF1) gene and also forms part of the "brain-lung-thyroid syndrome", in which additional developmental abnormalities of lung and thyroid tissue are observed. In this review, we summarize the ma...