Results 31 to 40 of about 287,910 (304)

Flow cytometry allows rapid detection of protein aggregates in cellular and zebrafish models of spinocerebellar ataxia 3

Disease Models & Mechanisms, 2021
Spinocerebellar ataxia 3 (SCA3, also known as Machado–Joseph disease) is a neurodegenerative disease caused by inheritance of a CAG repeat expansion within the ATXN3 gene, resulting in polyglutamine (polyQ) repeat expansion within the ataxin-3 protein ...
Katherine J. Robinson, Madelaine C. Tym, Alison Hogan, Maxinne Watchon, Kristy C. Yuan, Stuart K. Plenderleith, Emily K. Don, Angela S. Laird   +7 more
doaj   +1 more source

Tracking protein aggregate interactions [PDF]

Prion, 2011
Amyloid fibrils share a structural motif consisting of highly ordered β-sheets aligned perpendicular to the fibril axis ( 1, 2) . At each fibril end, β-sheets provide a template for recruiting and converting monomers ( 3) . Various amyloid fibrils often occur in the same individual, yet whether distinct protein aggregates aid or inhibit the assembly of
Nilsson, K. Peter R., Sigurdson, Christina J., Bartz, Jason C.   +2 more
openaire   +2 more sources

Oxidized protein aggregates

, 2020
The study of protein aggregates has a long history. While in the first decades until the 80ies of the 20th century only the observation of the presence of such aggregates was reported, later the biochemistry of the formation and the biological effects of
Grune, Tilman (Prof. Dr.)
core   +1 more source

Transcription Factor EB Activation Rescues Advanced αB‐Crystallin Mutation‐Induced Cardiomyopathy by Normalizing Desmin Localization

Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease, 2019
Background Mutations in αB‐crystallin result in proteotoxic cardiomyopathy with desmin mislocalization to protein aggregates. Intermittent fasting (IF) is a novel approach to activate transcription factor EB (TFEB), a master regulator of the autophagy ...
Xiucui Ma, Kartik Mani, Haiyan Liu, Attila Kovacs, John T. Murphy, Layla Foroughi, Brent A. French, Carla J. Weinheimer, Aldi Kraja, Ivor J. Benjamin, Joseph A. Hill, Ali Javaheri, Abhinav Diwan   +12 more
doaj   +1 more source

Distinct Mitochondrial Pathologies Caused by Mutations of the Proximal Tubular Enzymes EHHADH and GATM

Frontiers in Physiology, 2021
The mitochondria of the proximal tubule are essential for providing energy in this nephron segment, whose ATP generation is almost exclusively oxygen dependent.
Anna-Lena Forst, Markus Reichold, Robert Kleta, Richard Warth   +3 more
doaj   +1 more source

Imaging Protein Misfolding in the Brain Using β-Sheet Ligands

Frontiers in Neuroscience, 2018
Neurodegenerative diseases characterized by pathological protein accumulation in cells are termed “proteinopathies.” Although various protein aggregates share cross-β-sheet structures, actual conformations vary among each type of protein deposit.
Ryuichi Harada, Nobuyuki Okamura, Shozo Furumoto, Kazuhiko Yanai, Kazuhiko Yanai   +4 more
doaj   +1 more source

Integrated Omic Analysis Delineates Pathways Modulating Toxic TDP-43 Protein Aggregates in Amyotrophic Lateral Sclerosis

Cells, 2023
Amyotrophic lateral sclerosis (ALS) is a multi-systemic, incurable, amyloid disease affecting the motor neurons, resulting in the death of patients. The disease is either sporadic or familial with SOD1, C9orf72, FUS, and TDP-43 constituting the majority ...
Saiswaroop Rajaratnam, Akhil P. Soman, Kanikaram Sai Phalguna, Sai Sanwid Pradhan, Meghana Manjunath, Raksha Kanthavara Rao, Rajesh Babu Dandamudi, Sai Krishna Srimadh Bhagavatham, Sujith Kumar Pulukool, Sriram Rathnakumar, Sai Kocherlakota, Ashish Pargaonkar, Ravindra P. Veeranna, Natarajan Arumugam, Abdulrahman I. Almansour, Bibha Choudhary, Venketesh Sivaramakrishnan   +16 more
doaj   +1 more source

Impaired proteostatic mechanisms other than decreased protein synthesis limit old skeletal muscle recovery after disuse atrophy

Journal of Cachexia, Sarcopenia and Muscle, 2023
Background Skeletal muscle mass and strength diminish during periods of disuse but recover upon return to weight bearing in healthy adults but are incomplete in old muscle.
Jordan D. Fuqua, Marcus M. Lawrence, Zachary R. Hettinger, Agnieszka K. Borowik, Parker L. Brecheen, Marcelina M. Szczygiel, Claire B. Abbott, Frederick F. Peelor III, Amy L. Confides, Michael Kinter, Sue C. Bodine, Esther E. Dupont‐Versteegden, Benjamin F. Miller   +12 more
doaj   +1 more source

Folding and aggregation of designed proteins [PDF]

Proceedings of the National Academy of Sciences, 1998
Protein aggregation is studied by following the simultaneous folding of two designed identical 20-letter amino acid chains within the framework of a lattice model and using Monte Carlo simulations. It is found that protein aggregation is determined by elementary structures (partially folded intermediates) controlled by local contacts among some of the ...
Broglia R. A., Tiana G., Pasquali S., Roman H. E., Vigezzi E.   +4 more
openaire   +3 more sources

Broad and Complex Roles of NBR1-Mediated Selective Autophagy in Plant Stress Responses

Cells, 2020
Selective autophagy is a highly regulated degradation pathway for the removal of specific damaged or unwanted cellular components and organelles such as protein aggregates.
Yan Zhang, Zhixiang Chen
doaj   +1 more source