Results 41 to 50 of about 426,171 (247)

Integrating ER and Mitochondrial Proteostasis in the Healthy and Diseased Heart. [PDF]

Front Cardiovasc Med, 2019
The integrity of the proteome in cardiac myocytes is critical for robust heart function. Proteome integrity in all cells is managed by protein homeostasis or proteostasis, which encompasses processes that maintain the balance of protein synthesis, folding, and degradation in ways that allow cells to adapt to conditions that present a potential ...
Arrieta A, Blackwood EA, Stauffer WT, Glembotski CC.   +3 more
europepmc   +5 more sources

Mutant huntingtin disrupts mitochondrial proteostasis by interacting with TIM23. [PDF]

Proc Natl Acad Sci U S A, 2019
Significance We delineate the downstream pathologic consequences underlying the known mitochondrial protein import defect caused by mutant huntingtin (mHTT). We show direct high-affinity mHTT interaction with the inner mitochondrial membrane protein importing complex subunit translocase of mitochondrial inner membrane 23 (TIM23) and ...
Yablonska S, Ganesan V, Ferrando LM, Kim J, Pyzel A, Baranova OV, Khattar NK, Larkin TM, Baranov SV, Chen N, Strohlein CE, Stevens DA, Wang X, Chang YF, Schurdak ME, Carlisle DL, Minden JS, Friedlander RM.   +17 more
europepmc   +4 more sources

Coupling of autophagy and the mitochondrial intrinsic apoptosis pathway modulates proteostasis and ageing in Caenorhabditis elegans

Cell Death and Disease, 2023
Mitochondria preserve metabolic homeostasis and integrate stress signals, to trigger cytoprotective, or cell death pathways. Mitochondrial homeostasis and function decline with age. The mechanisms underlying the deterioration of mitochondrial homeostasis
Christina Ploumi, Emmanouil Kyriakakis, Nektarios Tavernarakis   +2 more
doaj   +1 more source

Increased mitochondrial protein import and cardiolipin remodelling upon early mtUPR.

PLoS Genetics, 2021
Mitochondrial defects can cause a variety of human diseases and protective mechanisms exist to maintain mitochondrial functionality. Imbalances in mitochondrial proteostasis trigger a transcriptional program, termed mitochondrial unfolded protein ...
Daniel Poveda-Huertes, Asli Aras Taskin, Ines Dhaouadi, Lisa Myketin, Adinarayana Marada, Lukas Habernig, Sabrina Büttner, F-Nora Vögtle   +7 more
doaj   +1 more source

Spatiotemporal Ca2+ nanodomain remodeling at MERCS regulates mitochondrial proteostasis. [PDF]

Protein Cell
Abstract Mitochondrial calcium fluxes serve as pivotal regulators of optimal organellar function and cellular viability, yet the spatiotemporal regulation of nanodomain Ca2+ transients at mitochondria–ER contact sites (MERCS) and their integration into adaptive mitochondrial stress signaling remain unresolved.
Lv Y, Zhao X, Li D, Hao Z, Zhao Y, Zhou Y, Zhang Y, Chen H, Lu Z, Li D, Guo Y.   +10 more
europepmc   +3 more sources

Mitochondrial proteostasis in the context of cellular and organismal health and aging. [PDF]

J Biol Chem, 2019
As a central hub of cellular metabolism and signaling, the mitochondrion is a crucial organelle whose dysfunction can cause disease and whose activity is intimately connected to aging. We review how the mitochondrial network maintains proteomic integrity, how mitochondrial proteotoxic stress is communicated and resolved in the context of the entire ...
Moehle EA, Shen K, Dillin A.
europepmc   +5 more sources

Tom70-based transcriptional regulation of mitochondrial biogenesis and aging

eLife, 2022
Mitochondrial biogenesis has two major steps: the transcriptional activation of nuclear genome-encoded mitochondrial proteins and the import of nascent mitochondrial proteins that are synthesized in the cytosol.
Qingqing Liu, Catherine E Chang, Alexandra C Wooldredge, Benjamin Fong, Brian K Kennedy, Chuankai Zhou   +5 more
doaj   +1 more source

Impaired age-associated mitochondrial translation is mitigated by exercise and PGC-1α [PDF]

, 2023
Sarcopenia, the age-related loss of skeletal muscle mass and function, can dramatically impinge on quality of life and mortality. While mitochondrial dysfunction and imbalanced proteostasis are recognized as hallmarks of sarcopenia, the regulatory and ...
Handschin, Christoph, Zavolan, Mihaela, de Smalen, Laura M., Gill, Jonathan F., Leuchtmann, Aurel B., Ritz, Danilo, Börsch, Anastasiya   +6 more
core   +1 more source

Mitochondrial Quality Control Strategies: Potential Therapeutic Targets for Neurodegenerative Diseases?

Frontiers in Neuroscience, 2021
Many lines of evidence have indicated the therapeutic potential of rescuing mitochondrial integrity by targeting specific mitochondrial quality control pathways in neurodegenerative diseases, such as Parkinson’s disease, Huntington’s disease, and ...
Di Hu, Zunren Liu, Xin Qi, Xin Qi
doaj   +1 more source

Disturb mitochondrial associated proteostasis: Neurodegeneration and imperfect ageing

Frontiers in Cell and Developmental Biology, 2023
The disturbance in mitochondrial functions and homeostasis are the major features of neuron degenerative conditions, like Parkinson’s disease, Amyotrophic Lateral Sclerosis, and Alzheimer’s disease, along with protein misfolding. The aberrantly folded proteins are known to link with impaired mitochondrial pathways, further contributing to disease ...
Yuvraj Anandrao Jagtap, Prashant Kumar, Sumit Kinger, Ankur Rakesh Dubey, Akash Choudhary, Ravi Kumar Gutti, Sarika Singh, Hem Chandra Jha, Krishna Mohan Poluri, Amit Mishra   +9 more
openaire   +3 more sources