Protein aggregation and prionopathies

• Published in: Pathologie biologie (Paris) Vol. 62; no. 3; pp. 162 - 168
• Main Authors: Renner, M., Melki, R.
• Format: Journal Article
• Language: English
• Published: Paris Elsevier SAS 01.06.2014; Elsevier; Elsevier Masson
• Subjects: Aging; Agrégation des protéines; Alpha-synucleinopathies; Alpha-synucléinopathies; Alzheimer; Alzheimer Disease; Alzheimer Disease - prevention & control; Amyotrophic Lateral Sclerosis; Amyotrophic Lateral Sclerosis - metabolism; Amyotrophic Lateral Sclerosis - pathology; Animals; Autophagy; Biological and medical sciences; Biopolymers; Cerebrospinal fluid. Meninges. Spinal cord; Clinical Trials, Phase II as Topic; Cognitive Sciences; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Disease Models, Animal; Drug Evaluation, Preclinical; Endocytosis; Humans; Huntington; Inclusion Bodies; Inclusion Bodies - chemistry; Inclusion Bodies - pathology; Life Sciences; Medical sciences; Mice; Nerve Tissue Proteins; Nerve Tissue Proteins - chemistry; Nervous system (semeiology, syndromes); Neurobiology; Neurodegenerative Diseases; Neurodegenerative Diseases - metabolism; Neurodegenerative Diseases - pathology; Neurofibrillary Tangles; Neurofibrillary Tangles - chemistry; Neurofibrillary Tangles - pathology; Neurology; Neurons and Cognition; Parkinson; Plaque, Amyloid; Plaque, Amyloid - chemistry; Plaque, Amyloid - pathology; Polysaccharides; Polysaccharides - therapeutic use; Prion; Prion Diseases; Prion Diseases - metabolism; Prion Diseases - pathology; Prion Diseases - veterinary; Prions; Prions - chemistry; Protein aggregation; Protein Aggregation, Pathological; Protein Aggregation, Pathological - metabolism; Protein Aggregation, Pathological - pathology; Protein Conformation; Psychology and behavior; Sclérose latérale amyotrophique; Solubility; Tauopathies; Tauopathies; Protein aggregation; Alpha-synucleinopathies; Agrégation des protéines; Amyotrophic lateral sclerosis; Prion; Huntington; Alpha-synucléinopathies; Sclérose latérale amyotrophique; Alzheimer; Parkinson; Aggregation; Nervous system diseases; Central nervous system disease; Degenerative disease; Spinal cord disease; Protein

Prion protein and prion-like proteins share a number of characteristics. From the molecular point of view, they are constitutive proteins that aggregate following conformational changes into insoluble particles. These particles escape the cellular clearance machinery and amplify by recruiting the so...