keyword
MENU ▼
Read by QxMD icon Read
search

Mitochondrial dysfunction neurodegenerative disease

keyword
https://www.readbyqxmd.com/read/28732510/protective-and-therapeutic-role-of-2-carba-cyclic-phosphatidic-acid-in-demyelinating-disease
#1
Shinji Yamamoto, Kota Yamashina, Masaki Ishikawa, Mari Gotoh, Sosuke Yagishita, Kensuke Iwasa, Kei Maruyama, Kimiko Murakami-Murofushi, Keisuke Yoshikawa
BACKGROUND: Multiple sclerosis is a neuroinflammatory demyelinating and neurodegenerative disease of the central nervous system characterized by recurrent and progressive demyelination/remyelination cycles, neuroinflammation, oligodendrocyte loss, demyelination, and axonal degeneration. Cyclic phosphatidic acid (cPA) is a natural phospholipid mediator with a unique cyclic phosphate ring structure at the sn-2 and sn-3 positions of the glycerol backbone. We reported earlier that cPA elicits a neurotrophin-like action and protects hippocampal neurons from ischemia-induced delayed neuronal death...
July 21, 2017: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/28724340/pharmacological-therapeutics-in-friedreich-ataxia-the-present-state
#2
Cassandra Strawser, Kimberly Schadt, Lauren Hauser, Ashley McCormick, Mckenzie Wells, Jane Larkindale, Hong Lin, David R Lynch
Friedreich ataxia (FRDA) is a progressive, inherited, neurodegenerative disease for which there is currently no cure or approved treatment. FRDA is caused by deficits in the production and expression of frataxin, a protein found in the mitochondria that is most likely responsible for regulating iron-sulfur cluster enzymes within the cell. A decrease in frataxin causes dysfunction of adenosine triphosphate synthesis, accumulation of mitochondrial iron, and other events leading to downstream cellular dysfunction...
July 20, 2017: Expert Review of Neurotherapeutics
https://www.readbyqxmd.com/read/28713939/microarray-analysis-reveals-key-genes-and-pathways-in-tetralogy-of-fallot
#3
Yue-E He, Hui-Xian Qiu, Jian-Bing Jiang, Rong-Zhou Wu, Ru-Lian Xiang, Yuan-Hai Zhang
The aim of the present study was to identify key genes that may be involved in the pathogenesis of Tetralogy of Fallot (TOF) using bioinformatics methods. The GSE26125 microarray dataset, which includes cardiovascular tissue samples derived from 16 children with TOF and five healthy age‑matched control infants, was downloaded from the Gene Expression Omnibus database. Differential expression analysis was performed between TOF and control samples to identify differentially expressed genes (DEGs) using Student's t‑test, and the R/limma package, with a log2 fold‑change of >2 and a false discovery rate of <0...
September 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28713289/mitochondrial-vdac1-a-key-gatekeeper-as-potential-therapeutic-target
#4
REVIEW
Amadou K S Camara, YiFan Zhou, Po-Chao Wen, Emad Tajkhorshid, Wai-Meng Kwok
Mitochondria are the key source of ATP that fuels cellular functions, and they are also central in cellular signaling, cell division and apoptosis. Dysfunction of mitochondria has been implicated in a wide range of diseases, including neurodegenerative and cardiac diseases, and various types of cancer. One of the key proteins that regulate mitochondrial function is the voltage-dependent anion channel 1 (VDAC1), the most abundant protein on the outer membrane of mitochondria. VDAC1 is the gatekeeper for the passages of metabolites, nucleotides, and ions; it plays a crucial role in regulating apoptosis due to its interaction with apoptotic and anti-apoptotic proteins, namely members of the Bcl-2 family of proteins and hexokinase...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28707399/neuroglobin-and-friends
#5
REVIEW
Marco Fiocchetti, Manuela Cipolletti, Valentina Brandi, Fabio Polticelli, Paolo Ascenzi
In the year 2000, the third member of the globin family was discovered in human and mouse brain and named neuroglobin (Ngb). Neuroglobin overexpression significantly protects both heart and brain from hypoxic/ischemic and oxidative stress-related insults, whereas decreased Ngb levels lead to an exacerbation of tissue injuries. Moreover, Ngb overexpression protects neurons from mitochondrial dysfunctions and neurodegenerative disorders such as Alzheimer disease; however, it facilitates the survival of cancer cells...
July 14, 2017: Journal of Molecular Recognition: JMR
https://www.readbyqxmd.com/read/28690763/tanshinone-iia-inhibits-glutamate-induced-oxidative-toxicity-through-prevention-of-mitochondrial-dysfunction-and-suppression-of-mapk-activation-in-sh-sy5y-human-neuroblastoma-cells
#6
Haifeng Li, Wenjing Han, Hongyu Wang, Fei Ding, Lingyun Xiao, Ruona Shi, Liping Ai, Zebo Huang
Glutamate excitotoxicity is associated with many neurological diseases, including cerebral ischemia and neurodegenerative diseases. Tanshinone IIA, a diterpenoid naphthoquinone from Salvia miltiorrhiza, has been shown to suppress presynaptic glutamate release, but its protective mechanism against glutamate-induced neurotoxicity is lacking. Using SH-SY5Y human neuroblastoma cells, we show here that excessive glutamate exposure decreases cell viability and proliferation and increases LDH release. Pretreatment with tanshinone IIA, however, prevents the decrease in cell viability and proliferation and the increase in LDH release induced by glutamate...
2017: Oxidative Medicine and Cellular Longevity
https://www.readbyqxmd.com/read/28676741/the-truncated-c-terminal-fragment-of-mutant-atxn3-disrupts-mitochondria-dynamics-in-spinocerebellar-ataxia-type-3-models
#7
Jung-Yu Hsu, Yu-Ling Jhang, Pei-Hsun Cheng, Yu-Fan Chang, Su-Han Mao, Han-In Yang, Chia-Wei Lin, Chuan-Mu Chen, Shang-Hsun Yang
Spinocerebellar ataxia type 3 (SCA3), known as Machado-Joseph disease, is an autosomal dominant disease caused by an abnormal expansion of polyglutamine in ATXN3 gene, leading to neurodegeneration in SCA3 patients. Similar to other neurodegenerative diseases, the dysfunction of mitochondria is observed to cause neuronal death in SCA3 patients. Based on previous studies, proteolytic cleavage of mutant ATXN3 is found to produce truncated C-terminal fragments in SCA3 models. However, whether these truncated mutant fragments disturb mitochondrial functions and result in pathological death is still unclear...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28674991/cell-death-mechanisms-of-neurodegeneration
#8
Jing Fan, Ted M Dawson, Valina L Dawson
There are common mechanisms shared by genetically or pathologically distinct neurodegenerative diseases, such as excitotoxicity, mitochondrial deficits and oxidative stress, protein misfolding and translational dysfunction, autophagy and microglia activation. This indicates that although the original cause may differ in individual diseases or even subtypes of certain disorders, these disrupted common cell functions and signaling, together with aging, may lead to final execution of cell death through similar pathways...
2017: Advances in Neurobiology
https://www.readbyqxmd.com/read/28669745/the-role-of-mitochondria-in-amyotrophic-lateral-sclerosis
#9
REVIEW
Emma F Smith, Pamela J Shaw, Kurt J De Vos
Mitochondria are unique organelles that are essential for a variety of cellular processes including energy metabolism, calcium homeostasis, lipid biosynthesis, and apoptosis. Mitochondrial dysfunction is a prevalent feature of many neurodegenerative diseases including motor neuron disorders such as amyotrophic lateral sclerosis (ALS). Disruption of mitochondrial structure, dynamics, bioenergetics and calcium buffering has been extensively reported in ALS patients and model systems and has been suggested to be directly involved in disease pathogenesis...
June 29, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28668382/inflammation-and-mitochondrial-dysfunction-a-vicious-circle-in-neurodegenerative-disorders
#10
REVIEW
Jack van Horssen, Pauline van Schaik, Maarten Witte
Experimental evidence supports an intricate association between inflammation and mitochondrial dysfunction as main contributors of neurological diseases. Inflammatory mediators produced by activated microglia and infiltrated immune cells trigger intracellular signaling cascades that can alter cellular mitochondrial metabolism. Cytokines, particularly tumor necrosis factor-alpha, impede mitochondrial oxidative phosphorylation and associated ATP production and instigate mitochondrial reactive oxygen species production...
June 28, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28666219/lipid-induced-endoplasmic-reticulum-stress-in-x-linked-adrenoleukodystrophy
#11
Malu-Clair van de Beek, Rob Ofman, Inge Dijkstra, Frits Wijburg, Marc Engelen, Ronald Wanders, Stephan Kemp
X-linked adrenoleukodystrophy (ALD) is a progressive neurodegenerative disease that is caused by mutations in the ABCD1 gene and characterized by elevated levels of very long-chain fatty acids (VLCFA) in plasma and tissues, with the most pronounced increase in the central nervous system. Virtually all male patients develop adrenal insufficiency and myelopathy (adrenomyeloneuropathy), but a subset develops a fatal cerebral demyelinating disease (known as cerebral ALD). Female patients may also develop myelopathy, but adrenal insufficiency or leukodystrophy are very rare...
June 27, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28659480/mitochondrial-respiration-is-impaired-during-late-stage-hamster-prion-infection
#12
Robert Faris, Roger A Moore, Anne Ward, Dan E Sturdevant, Suzette A Priola
Mitochondria are crucial to proper neuronal function and overall brain health. Mitochondrial dysfunction within the brain has been observed in many neurodegenerative diseases including prion disease. Several markers of decreased mitochondrial activity have been reported during prion infection yet the bioenergetic respiratory status of mitochondria from prion-infected animals is unknown. Here, we show that clinically ill transgenic mice overexpressing hamster prion protein (Tg7) infected with the hamster prion strain 263K suffer from a severe deficit in mitochondrial oxygen consumption in response to the respiratory Complex II substrate, succinate...
June 28, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28658984/cedrin-identified-from-cedrus-deodara-roxb-g-don-protects-pc12-cells-against-neurotoxicity-induced-by-a%C3%AE-1-42
#13
Zhiwei Zhao, Zhanfei Dong, Jie Ming, Yan Liu
Alzheimer's disease is a severe neurodegenerative disease affecting elder worldwide and closely related to the neurotoxicity induced by amyloid β. To find efficient therapeutics, we have investigated the protective effects of cedrin from Cedrus deodara (Roxb.) G. Don on PC12 cells against the neurotoxicity induced by amyloid β1-42. The results have shown the viability of PC12 cells injured by amyloid β1-42 can be improved by cedrin. Cedrin can reduce reacrive oxygen species overproduction, increase the activity of superoxide dismutase and decrease malondialdehyde content...
June 29, 2017: Natural Product Research
https://www.readbyqxmd.com/read/28654243/mitochondria-targeted-triphenylphosphonium-based-compounds-syntheses-mechanisms-of-action-and-therapeutic-and-diagnostic-applications
#14
Jacek Zielonka, Joy Joseph, Adam Sikora, Micael Hardy, Olivier Ouari, Jeannette Vasquez-Vivar, Gang Cheng, Marcos Lopez, Balaraman Kalyanaraman
Mitochondria are recognized as one of the most important targets for new drug design in cancer, cardiovascular, and neurological diseases. Currently, the most effective way to deliver drugs specifically to mitochondria is by covalent linking a lipophilic cation such as an alkyltriphenylphosphonium moiety to a pharmacophore of interest. Other delocalized lipophilic cations, such as rhodamine, natural and synthetic mitochondria-targeting peptides, and nanoparticle vehicles, have also been used for mitochondrial delivery of small molecules...
June 27, 2017: Chemical Reviews
https://www.readbyqxmd.com/read/28652416/mitochondrial-membrane-dynamics-and-inherited-optic-neuropathies
#15
REVIEW
Eleni Bagli, Anastasia K Zikou, Niki Agnantis, Georgios Kitsos
Inherited optic neuropathies are a genetically diverse group of disorders mainly characterized by visual loss and optic atrophy. Since the first recognition of Leber's hereditary optic neuropathy, several genetic defects altering primary mitochondrial respiration have been proposed to contribute to the development of syndromic and non-syndromic optic neuropathies. Moreover, the genomics and imaging revolution in the past decade has increased diagnostic efficiency and accuracy, allowing recognition of a link between mitochondrial dynamics machinery and a broad range of inherited neurodegenerative diseases involving the optic nerve...
July 2017: In Vivo
https://www.readbyqxmd.com/read/28649128/lx2343-alleviates-cognitive-impairments-in-ad-model-rats-by-inhibiting-oxidative-stress-induced-neuronal-apoptosis-and-tauopathy
#16
Xiao-Dan Guo, Guang-Long Sun, Ting-Ting Zhou, Yi-Yang Wang, Xin Xu, Xiao-Fan Shi, Zhi-Yuan Zhu, Vatcharin Rukachaisirikul, Li-Hong Hu, Xu Shen
Alzheimer's disease (AD) is a progressive neurodegenerative disease leading to the irreversible loss of brain neurons and cognitive abilities, and the vicious interplay between oxidative stress (OS) and tauopathy is believed to be one of the major players in AD development. Here, we demonstrated the capability of the small molecule N-(1,3-benzodioxol-5-yl)-2-[5-chloro-2-methoxy(phenylsulfonyl)anilino]acetamide (LX2343) to ameliorate the cognitive dysfunction of AD model rats by inhibiting OS-induced neuronal apoptosis and tauopathy...
June 26, 2017: Acta Pharmacologica Sinica
https://www.readbyqxmd.com/read/28644490/potentiating-role-of-copper-on-spatial-memory-deficit-induced-by-beta-amyloid-and-evaluation-of-mitochondrial-function-markers-in-the-hippocampus-of-rats
#17
Ladan Behzadfar, Mohammad Abdollahi, Omid Sabzevari, Rohollah Hosseini, Ahmad Salimi, Parvaneh Naserzadeh, Mohammad Sharifzadeh, Jalal Pourahmad
Mounting evidence suggests that copper, a crucial element in normal brain function, plays an important role in the etiology of Alzheimer's disease, which is known as a neurodegenerative mitochondrial disorder. However, the precise mechanisms of its effects on cognitive and mitochondrial functions through the CNS have not been thoroughly recognized yet. In this study, we aimed to investigate the long-term (3-week) effects of copper sulfate (50, 100 and 200 mg kg(-1) day(-1)) exposure on learning and memory as well as on mitochondrial function in the hippocampus of rats in the presence and absence of beta amyloid (1 μg μl(-1) per side) intrahippocampally (IH)...
July 19, 2017: Metallomics: Integrated Biometal Science
https://www.readbyqxmd.com/read/28639241/huntington-s-disease-and-mitochondria
#18
REVIEW
Mohammad Jodeiri Farshbaf, Kamran Ghaedi
Huntington's disease (HD) as an inherited neurodegenerative disorder leads to neuronal loss in striatum. Progressive motor dysfunction, cognitive decline, and psychiatric disturbance are the main clinical symptoms of the HD. This disease is caused by expansion of the CAG repeats in exon 1 of the huntingtin which encodes Huntingtin protein (Htt). Various cellular and molecular events play role in the pathology of HD. Mitochondria as important organelles play crucial roles in the most of neurodegenerative disorders like HD...
June 21, 2017: Neurotoxicity Research
https://www.readbyqxmd.com/read/28638596/environmental-insults-critical-triggers-for-amyotrophic-lateral-sclerosis
#19
REVIEW
Bing Yu, Roger Pamphlett
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterised by a rapid loss of lower and upper motor neurons. As a complex disease, the ageing process and complicated gene-environment interactions are involved in the majority of cases. MAIN BODY: Significant advances have been made in unravelling the genetic susceptibility to ALS with massively parallel sequencing technologies, while environmental insults remain a suspected but largely unexplored source of risk...
2017: Translational Neurodegeneration
https://www.readbyqxmd.com/read/28627426/neurodegenerative-signaling-factors-and-mechanisms-in-parkinson-s-pathology
#20
REVIEW
Poonam Goswami, Neeraj Joshi, Sarika Singh
Parkinson's disease (PD) is a chronic and progressive degenerative disorder of central nervous system which is mainly characterized by selective loss of dopaminergic neurons in the nigrostrial pathway. Clinical symptoms of this devastating disease comprise motor impairments such as resting tremor, bradykinesia, postural instability and rigidity. Current medications only provide symptomatic relief but fail to halt the dopaminergic neuronal death. While the etiology of dopaminergic neuronal death is not fully understood, combination of various molecular mechanisms seems to play a critical role...
June 13, 2017: Toxicology in Vitro: An International Journal Published in Association with BIBRA
keyword
keyword
107610
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"