...Mohammed M. Alhadidy; Nicholas M. Kanaan Tau protein is associated with many neurodegenerative disorders known as tauopathies. Aggregates of tau are thought of as a main contributor to neurodegeneration in these diseases. Increasingly, evidence points to earlier, soluble conformations of abnormally...

... ]. aggregation amyloid prion protein misfolding In contrast, different patients with systemic AL amyloidosis and strong cardiomyopathy can be associated with rather different fibril morphologies [ 10 , 19 , 20 ], as there are the substantial patient-specific variations in the primary structures...

.... The aggregation-prone LSCRs (low-sequence-complexity regions), and in particular, the polyQ/N-rich regions, have been extensively studied under pathological conditions due to their role in neurodegenerative diseases. In the present review, we discuss recent in vitro , in vivo and computational data that, globally...

... of brain inclusions in ALS (amyotrophic lateral sclerosis) and FTLD (frontotemporal lobar degeneration) patients. Functionally, this aberrant aggregation and mislocalization implies that, in the affected neurons, transcripts regulated by TDP-43 may be altered. Since then, a considerable amount of data has...

...Yipeng Wang; Eckhard Mandelkow Tau aggregates are present in several neurodegenerative diseases and correlate with the severity of memory deficit in AD (Alzheimer's disease). However, the triggers of tau aggregation and tau-induced neurodegeneration are still elusive. The impairment of protein...

... Balschun; Rudi D'Hooge; Malika Hamdane; David Blum; Nicolas Sergeant Tau pathology is characterized by intracellular aggregates of abnormally and hyperphosphorylated tau proteins. It is encountered in many neurodegenerative disorders, but also in aging. These neurodegenerative disorders are referred...

...Yipeng Wang; Sarika Garg; Eva-Maria Mandelkow; Eckhard Mandelkow Tau aggregation is a hallmark of several neurodegenerative diseases, including AD (Alzheimer's disease), although the mechanism underlying tau aggregation remains unclear. Recent studies show that the proteolysis of tau plays...

...Mark R.H. Krebs; Kristin R. Domike; Athene M. Donald The aggregation of misfolded proteins into amyloid fibrils, and the importance of this step for various diseases, is well known. However, it is becoming apparent that the fibril is not the only structure that aggregating proteins of widely...

... ). 28 10 2008 © The Authors Journal compilation © 2009 Biochemical Society 2009 aggregation archaeon DNA repair hyperthermophile Sulfolobus UV damage All living organisms, from bacteria to archaea and eukaryotes, have an impressive number of proteins and pathways that seem...

... conditions in order to obtain reliable conditions for producing fibrils for further structural investigations. The results indicate that the rate of aggregation and the morphology of the fibrils formed are sensitive to pH and temperature. Amyloid fibril formation is thought to be a central event...

... or delayed clearance of protein aggregates. Hence, inhibition of pathologic protein–protein interactions is a very attractive mechanism for drug development. This review focuses on a novel therapeutic strategy to inhibit the de novo formation of protein aggregates. Inspired by strategies used in Nature...

...J. Madine; A.J. Doig; D.A. Middleton α-Synuclein is a 140 amino acid protein, which is associated with presynaptic membranes in the brain, and is the major component of protein aggregates produced during the progression of many neurodegenerative diseases. It has been shown that a central...

... and aggregation. Detailed understanding of these phenomena is therefore crucial in order to rationalize different phases of the diseases. In the past decade, a few studies have focused on the structural properties of polyglutamine and on the molecular bases of the aggregation process. Most of these studies have...

..., which has a well-recognized function in gene regulation, provides a unique opportunity to investigate the functional significance of poly(amino acid) repeats in normal and disease states. 1 e-mail: [email protected] 21 12 2000 © 2001 Biochemical Society 2001 aggregation...