Mitochondrial dysfunction and biogenesis in neurodegenerative diseases: pathogenesis and treatment

M Golpich, E Amini, Z Mohamed… - CNS neuroscience & …, 2017 - Wiley Online Library
Neurodegenerative diseases are a heterogeneous group of disorders that are incurable and
characterized by the progressive degeneration of the function and structure of the central …

Neuronal lipid metabolism: multiple pathways driving functional outcomes in health and disease

TJ Tracey, FJ Steyn, EJ Wolvetang… - Frontiers in molecular …, 2018 - frontiersin.org
Lipids are a fundamental class of organic molecules implicated in a wide range of biological
processes related to their structural diversity, and based on this can be broadly classified …

TDP-43 aggregation induced by oxidative stress causes global mitochondrial imbalance in ALS

X Zuo, J Zhou, Y Li, K Wu, Z Chen, Z Luo… - Nature structural & …, 2021 - nature.com
Amyotrophic lateral sclerosis (ALS) was initially thought to be associated with oxidative
stress when it was first linked to mutant superoxide dismutase 1 (SOD1). The subsequent …

[HTML][HTML] Osteocalcin signaling in myofibers is necessary and sufficient for optimum adaptation to exercise

P Mera, K Laue, M Ferron, C Confavreux, J Wei… - Cell metabolism, 2016 - cell.com
Circulating levels of undercarboxylated and bioactive osteocalcin double during aerobic
exercise at the time levels of insulin decrease. In contrast, circulating levels of osteocalcin …

PPARγ/PGC1α signaling as a potential therapeutic target for mitochondrial biogenesis in neurodegenerative disorders

S Jamwal, JK Blackburn, JD Elsworth - Pharmacology & Therapeutics, 2021 - Elsevier
Neurodegenerative diseases represent some of the most devastating neurological
disorders, characterized by progressive loss of the structure and function of neurons. Current …

Motor neuron vulnerability and resistance in amyotrophic lateral sclerosis

J Nijssen, LH Comley, E Hedlund - Acta neuropathologica, 2017 - Springer
In the fatal disease—amyotrophic lateral sclerosis (ALS)—upper (corticospinal) motor
neurons (MNs) and lower somatic MNs, which innervate voluntary muscles, degenerate …

PGC1α and mitochondrial metabolism–emerging concepts and relevance in ageing and neurodegenerative disorders

S Austin, J St-Pierre - Journal of cell science, 2012 - journals.biologists.com
PGC1α is a transcriptional coactivator that is a central inducer of mitochondrial biogenesis in
cells. Recent work highlighted that PGC1α can also modulate the composition and functions …

New insights into PGC‐1 coactivators: redefining their role in the regulation of mitochondrial function and beyond

JA Villena - The FEBS journal, 2015 - Wiley Online Library
Members of the PGC‐1 family of coactivators have been revealed as key players in the
regulation of energy metabolism. Early gain‐and loss‐of‐function studies led to the …

Neuromuscular junction dysfunction in amyotrophic lateral sclerosis

S Verma, S Khurana, A Vats, B Sahu, NK Ganguly… - Molecular …, 2022 - Springer
Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder characterized by
progressive degeneration of motor neurons leading to skeletal muscle denervation. Earlier …

Mitochondrial diseases of the brain

RK Chaturvedi, MF Beal - Free Radical Biology and Medicine, 2013 - Elsevier
Neurodegenerative disorders are debilitating diseases of the brain, characterized by
behavioral, motor and cognitive impairments. Ample evidence underpins mitochondrial …