Results 101 to 110 of about 7,354 (190)

Metformin rescues muscle function in BAG3 myofibrillar myopathy models

, 2020
Dominant de novo mutations in the co-chaperone BAG3 cause a severe form of myofibrillar myopathy, exhibiting progressive muscle weakness, muscle structural failure, and protein aggregation. To elucidate the mechanism of disease in, and identify therapies
Emmanuelle Lacene (6177269), Emily C. Baxter (9491444), Tanya Stojkovic (445701), Keyne Monro (2924136), Viola Oorschot (113700), Yvan Torrente (54046), Clara Lee (2557852), Caitlin Williams (2069830), Mirella Meregalli (141757), Keith E. Schulze (6895205), Robert J. Bryson-Richardson (7907198), Rita J. Serrano (9491441), Avnika A. Ruparelia (4839585), Margit Fuchs (817677), Georg Ramm (173555), Josée N. Lavoie (9491447), Emily A. McKaige (4839582)   +16 more
core   +1 more source

BAG3 mRNA is present in synaptosomal polysomes of rat brain

, 2014
BAG3 is a 75-kDa protein of the family of co-chaperones that interacts with the ATPase domain of heat shock protein Hsp70 through the BAG domain.
ROSATI, ALESSANDRA, CRISPINO, MARIANNA, Anna Paola Bruno, Alessandra Rosati, Carolina Cefaliello, D'AURIA, Raffaella, NORI, Stefania Lucia, BRUNO, ANNA PAOLA, Stefania Lucia Nori, Marianna Crispino, Antonio Giuditta, Raffaella D'Auria   +11 more
core   +1 more source

BAG3-mediated proteostasis at a glance

, 2017
Cellular and organismal survival depend on the ability to maintain the proteome, even under conditions that threaten protein integrity. BCL2-associated athanogene 3 (BAG3) is essential for protein homeostasis (proteostasis) in stressed cells.
Jörg Höhfeld, Christina Klimek, Barbara Kathage, Judith Wördehoff   +3 more
core   +1 more source

Myopathy associated BAG3 mutations lead to protein aggregation by stalling Hsp70 networks

Nature Communications, 2018
BAG3 is a Hsp70 co-chaperone that is highly expressed in muscles. Here the authors show that several myofibrillar myopathy causing BAG3 mutations are not impaired in Hsp70 binding, but rather impair the ADP-ATP exchange step of the Hsp70 cycle, causing ...
Melanie Meister-Broekema, Rebecca Freilich, Chandhuru Jagadeesan, Jennifer N. Rauch, Rocio Bengoechea, William W. Motley, E. F. Elsiena Kuiper, Melania Minoia, Gabriel V. Furtado, Maria A. W. H. van Waarde, Shawn J. Bird, Adriana Rebelo, Stephan Zuchner, Peter Pytel, Steven S. Scherer, Federica F. Morelli, Serena Carra, Conrad C. Weihl, Steven Bergink, Jason E. Gestwicki, Harm H. Kampinga   +20 more
doaj   +1 more source

The Role of the Multifunctional BAG3 Protein in Cellular Protein Quality Control and in Disease

Frontiers in Molecular Neuroscience, 2017
In neurons, but also in all other cells the complex proteostasis network is monitored and tightly regulated by the cellular protein quality control (PQC) system. Beyond folding of newly synthesized polypeptides and their refolding upon misfolding the PQC
Elisabeth Stürner, Christian Behl
doaj   +1 more source

Tuning the B-CLL microenvironment: evidence for BAG3 protein- mediated regulation of stromal fibroblasts activity

Cell Death Discovery
The Bcl2-associated athanogene-3 (BAG3) protein, a critical regulator of cellular survival, has been identified as a potential therapeutic target in various malignancies.
Anna Basile, Valentina Giudice, Laura Mettivier, Antonia Falco, Anna Lisa Cammarota, Angela D’Ardia, Carmine Selleri, Margot De Marco, Nicola De Maio, Maria Caterina Turco, Liberato Marzullo, Alessandra Rosati   +11 more
doaj   +1 more source

Generation of human induced pluripotent stem cell (iPSC) lines from two patients with BAG3 P209L myofibrillar myopathy-6.

Stem Cell Research
As member of the chaperone-assisted selective autophagy (CASA) complex, BAG3 is important for the turnover of muscle proteins. Patients with a point mutation at position 626 in the BAG3 gene (p.P209L, c.626C>T, Chr.10q26) suffer from polyneuropathy and ...
Kerstin Filippi, Martin Wiemann, Stefan Rupp, Michael Peitz, Bernd K. Fleischmann, Michael Hesse   +5 more
semanticscholar   +1 more source

Familial hypertrophic cardiomyopathy with midventricular obstruction associated with MYH7, FHOD3 and BAG3 gene variants: a case report

Российский кардиологический журнал
Introduction. Hypertrophic cardiomyopathy (HCM) with midventricular obstruction (MVO) represents a subgroup of increased risk of adverse outcomes. It contributes to development of apical aneurysm, which is an anatomical substrate for the development of ...
S. E. Andreeva, M. O. Marusova, M. A. Bortsova, A. A. Kostareva   +3 more
doaj   +1 more source

Synthetic lethality-based approach to uncover BAG3-related vulnerabilities in Glioblastoma Multiforme

reservedIl glioblastoma multiforme è il tumore cerebrale maligno più comune negli adulti e rappresenta una delle neoplasie più aggressive.
PORTALURI, MARTINA
core  

bag3 gene expression is regulated by heat shock factor 1.

, 2008
BAG3 protein, a member of the BAG co-chaperones family, sustains cell survival, through its interaction with the heat shock protein (HSP) 70, in a variety of normal and neoplastic cell types. bag3 gene expression is induced by stressful stimuli.
ROSATI, ALESSANDRA, DE NICOLA S, FRANCESCHELLI, Silvia, TURCO, Maria Caterina, LEROSE R, PASCALE, Maria   +5 more
core   +1 more source