Mitochondrial unfolded protein response: An emerging pathway in human diseases

L Zhu, Q Zhou, L He, L Chen - Free Radical Biology and Medicine, 2021 - Elsevier
Mitochondrial unfolded protein response (UPR mt) is a mitochondria stress response, which
the transcriptional activation programs of mitochondrial chaperone proteins and proteases …

The different axes of the mammalian mitochondrial unfolded protein response

C Münch - BMC biology, 2018 - Springer
Mitochondria are sensitive to numerous environmental stresses, which can lead to activation
of mitochondrial stress responses (MSRs). Of particular recent interest has been the …

[HTML][HTML] Nrf2 deficiency exacerbates age-related contractile dysfunction and loss of skeletal muscle mass

B Ahn, G Pharaoh, P Premkumar, K Huseman, R Ranjit… - Redox biology, 2018 - Elsevier
Age-related loss of skeletal muscle mass and contractile dysfunction, or sarcopenia, reduces
independence and quality of life in the elderly and leads to increased risk of comorbidities …

[HTML][HTML] Insulin-like growth factor receptor signaling regulates working memory, mitochondrial metabolism, and amyloid-β uptake in astrocytes

S Logan, GA Pharaoh, MC Marlin, DR Masser… - Molecular …, 2018 - Elsevier
Objective A decline in mitochondrial function and biogenesis as well as increased reactive
oxygen species (ROS) are important determinants of aging. With advancing age, there is a …

Tissue‐ and Condition‐Specific Isoforms of Mammalian Cytochrome c Oxidase Subunits: From Function to Human Disease

CA Sinkler, H Kalpage, J Shay, I Lee… - Oxidative medicine …, 2017 - Wiley Online Library
Cytochrome c oxidase (COX) is the terminal enzyme of the electron transport chain and
catalyzes the transfer of electrons from cytochrome c to oxygen. COX consists of 14 subunits …

mtUPR modulation as a therapeutic target for primary and secondary mitochondrial diseases

P Cilleros-Holgado, D Gómez-Fernández… - International Journal of …, 2023 - mdpi.com
Mitochondrial dysfunction is a key pathological event in many diseases. Its role in energy
production, calcium homeostasis, apoptosis regulation, and reactive oxygen species (ROS) …

Metabolic and stress response changes precede disease onset in the spinal cord of mutant SOD1 ALS mice

G Pharaoh, K Sataranatarajan, K Street, S Hill… - Frontiers in …, 2019 - frontiersin.org
Many Amyotrophic Lateral Sclerosis (ALS) patients experience hypermetabolism, or an
increase in measured vs. calculated metabolic rate. The cause of hypermetabolism and the …

Mitochondria: intracellular sentinels of infections

D Brokatzky, G Häcker - Medical Microbiology and Immunology, 2022 - Springer
Abstract Structure and integrity of the mitochondrial network play important roles in many
cellular processes. Loss of integrity can lead to the activation of a variety of signalling …

[Retracted] Mitochondrial ROS Produced by Skeletal Muscle Mitochondria Promote the Decisive Signal for UPRmt Activation

Z Wang, H Bo, Y Song, C Li… - BioMed Research …, 2022 - Wiley Online Library
The mitochondrial unfolded protein response (UPRmt) can repair and remove misfolded or
unfolded proteins in mitochondria and enhance mitochondrial protein homeostasis …

Role of signaling molecules in mitochondrial stress response

S Hill, K Sataranatarajan, H Van Remmen - Frontiers in genetics, 2018 - frontiersin.org
Mitochondria are established essential regulators of cellular function and metabolism.
Mitochondria regulate redox homeostasis, maintain energy (ATP) production through …