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Mitochondrial Dysfunction in Huntington Disease
Published in Abhai Kumar, Debasis Bagchi, Antioxidants and Functional Foods for Neurodegenerative Disorders, 2021
Md. Hafiz Uddin, Marufa Rumman, Tasnuva Sarowar
Mitochondrial dysfunction is a common feature in neurodegenerative disorders as well as aging and other diseases (Velarde 2014). There is sufficient evidence of the association of mitochondrial dysfunction in HD (Petrozzi et al. 2007), usually due to the imbalance of ROS production, changes in the mitochondrial dynamics, the levels/activity of antioxidant defenses, etc. (Sohal and Allen 1990). An overview of mutant HTT (mHTT)-associated mitochondrial dysfunction in HD shown in Figure 9.1. The high energy demands of the neuron are mostly met by mitochondrial OXPHOS as neurons have a limited capacity to utilize energy generated from glycolysis (Herrero-Mendez et al. 2009). Hence, neurons are mostly unprotected to mitochondrial dysfunction. Mitochondrial failure leads to synaptic dysfunction, protein aggregation, brain atrophy, and loss of function (Gonzalez-Lima, Barksdale, Rojas 2014). A recent study showed that mutations in mtDNA at birth can accelerate aging in animals even when the nuclear genomes are normal (Ross et al. 2013), suggesting the involvement of maternally inherited mtDNA variants in age-related disorders. The most important mechanisms associated with mitochondrial anomalies are illustrated in several topics below.
Degenerative Diseases of the Nervous System
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
James A. Mastrianni, Elizabeth A. Harris
Metabolic: Calcifications:57 Fahr's disease or other disturbances of calcium metabolism (hypo- and hyperparathyroidism, pseudohypoparathyroidism).Mitochondrial disease: Leigh's disease (subacute necrotizing encephalopathy).Mitochondrial cytopathies with striatal necrosis.
The cell and tissues
Published in Peate Ian, Dutton Helen, Acute Nursing Care, 2020
Mitochondria also contain DNA (mtDNA) which is almost exclusively inherited from the mother. It can be very significant in the development of disease, partly because the quantity of it in the body means that it is subject to significant mutation (McCance and Heuther 2018).
Identification of Rab7 as an autophagy marker: potential therapeutic approaches and the effect of Qi Teng Xiao Zhuo granule in chronic glomerulonephritis
Published in Pharmaceutical Biology, 2023
Xiujuan Qin, Huiyu Chen, Xiaoli Zhu, Xianjin Xu, Jiarong Gao
Mitochondria are important eukaryotic cell organelles; they produce ATP via oxidative phosphorylation and provide 95% of the cell’s energy requirements. They are also involved in metabolic signal transduction, inflammation, and apoptosis regulation. The kidney is rich in mitochondria, which play a key role in its function, and mitochondrial damage and dysfunction are major factors in many chronic and acute kidney diseases (Tang et al. 2021). Maintaining mitochondrial homeostasis and metabolic balance is crucial for kidney function (Bhargava and Schnellmann 2017). When mitochondrial damage and dysfunction occur, mitophagy is induced to maintain cell homeostasis, removing damaged or excess mitochondria (Su et al. 2023). Transmission electron microscopy showed that abnormal mitochondrial cristae and decreased autophagosomes were apparent in the model group. Interestingly, we also found that mitochondrial damage was reduced after QTXZG treatment.
Syntaphilin mediates axonal growth and synaptic changes through regulation of mitochondrial transport: a potential pharmacological target for neurodegenerative diseases
Published in Journal of Drug Targeting, 2023
Qing-Yun Wu, Hui-Lin Liu, Hai-Yan Wang, Kai-Bin Hu, Ping Liao, Sen Li, Zai-Yun Long, Xiu-Min Lu, Yong-Tang Wang
Physiological activities such as the generation of nerve impulses, the formation of synapses, and the transmission of nerve signalling are all heavily energy-consuming processes. Mitochondria, the organelles found in eukaryotic cells, are responsible for converting stored energy from organic matter into adenosine triphosphate (ATP). They play a critical role in cellular energy metabolism and produce 90% of the ATP required for cellular metabolism [1]. The brain relies heavily on mitochondria to produce most of the ATP needed for its functions and energy metabolism [2], and synapses are the main site of energy expenditure [3]. As the primary energy source for neurons, mitochondria are crucial for maintaining synaptic activities, including synaptic assembly, action potential and synaptic potential production, and synaptic vesicle (SV) transport and recycling [4]. Axonal mitochondrial deficiency affects synaptic transmission, and defective mitochondrial transport and energy deficiency are associated with the failure of axonal regeneration after injury and the pathogenesis of multiple neurological diseases [5–7]. Mitochondrial motility is also affected by stress or damage to its integrity. Consequently, ensuring mitochondrial health and motor function is essential for axonal growth, maintenance of synaptic energy balance, and synaptic function.
Development of novel 9-O-substituted-13-octylberberine derivatives as potential anti-hepatocellular carcinoma agents
Published in Journal of Enzyme Inhibition and Medicinal Chemistry, 2022
Jichao Chen, Yiping Duan, Xiaoxuan Yu, Jiarou Zhong, Jing Bai, Nian-Guang Li, Zheying Zhu, Jinyi Xu
It is well known that mitochondria play an important role in the process of apoptosis. Mitochondrial dysfunction triggers an alteration in mitochondrial structure, which can result in disruption of mitochondrial membrane potential (MMP) to initiate the apoptosis pathway28. In order to explore whether 6k could induce mitochondrial dysfunction, the lipophilic mitochondrial probe JC-1 was employed to measure MMP. As shown in Figure 3(C,D), treatment with 6k at concentrations from 0.25 to 1.0 μM for 72 h, the percentages of HepG2 cells with green fluorescence intensity correspondingly increased from 10.33% to 37.52%, as compared with 1.90% in the control group, suggesting that 6k caused MMP collapse and mitochondrial dysfunction in the process of HepG2 apoptosis.