NR ACHC
AU Carrell,R.W.; Lomas,D.A.
TI Conformational disease
QU Lancet 1997 Jul 12; 350(9071): 134-8
KI Lancet. 2000 Feb 19;355(9204):590-1. PMID: 10696974
PT journal article; review; review, tutorial
AB Several diverse disorders, including the prevalent dementias and encephalopathies, are now believed to arise from the same general disease mechanism. In each, there is abnormal unfolding and then aggregation of an underlying protein. The gradual accumulation of these aggregates and the acceleration of their formation by stress explain the characteristic late or episodic onset of the clinical disease. The understanding of these processes at the molecular level is opening prospects of more rational approaches to investigation and therapy.
IN Die Autoren schlagen den Begriff Konformationskrankheiten für Krankheiten vor, bei denen die posttranslationale Änderung der Tertiärstruktur zur Aggregation und Akkumulation des veränderten Proteins führt. Zu dieser Famlie zählen sie neben den Prion-Krankheiten
ZR 40
MH Amyloid/chemistry; Brain Diseases/*etiology/genetics/therapy; Dementia/etiology; Human; Molecular Biology; Prions/chemistry; *Protein Conformation; Protein Folding; Protein Structure, Secondary; Proteins/chemistry/genetics; Serpins/chemistry; Stress/physiopathology; alpha 1-Antitrypsin/chemistry
AD Robin W. Carrell (rwc1000@cam.ac.uk), Department of Haematology, University of Cambridge, MRC Centre, Addenbrooke's Hospital, UK
SP englisch
PO England
OR Prion-Krankheiten C