keyword
MENU ▼
Read by QxMD icon Read
search

Mitochondrial dysfunction neurodegenerative disease

keyword
https://www.readbyqxmd.com/read/29767814/inborn-errors-of-coenzyme-a-metabolism-and-neurodegeneration
#1
Ivano Di Meo, Miryam Carecchio, Valeria Tiranti
Two inborn errors of coenzyme A (CoA) metabolism are responsible for distinct forms of neurodegeneration with brain iron accumulation (NBIA), a heterogeneous group of neurodegenerative diseases having as a common denominator iron accumulation mainly in the inner portion of globus pallidus. Pantothenate kinase-associated neurodegeneration (PKAN), an autosomal recessive disorder with progressive impairment of movement, vision and cognition, is the most common form of NBIA and is caused by mutations in the pantothenate kinase 2 gene (PANK2), coding for a mitochondrial enzyme, which phosphorylates vitamin B5 in the first reaction of the CoA biosynthetic pathway...
May 16, 2018: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29732581/discrete-mitochondrial-aberrations-in-the-spinal-cord-of-sporadic-als-patients
#2
Vedad Delic, Crupa Kurien, Josean Cruz, Sandra Zivkovic, Jennifer Barretta, Avery Thomson, Daniel Hennessey, Jaheem Joseph, Jared Ehrhart, Alison E Willing, Patrick Bradshaw, Svitlana Garbuzova-Davis
Amyotrophic lateral sclerosis (ALS) is an adult onset neurodegenerative disease characterized by progressive motor neuron degeneration in the brain and spinal cord leading to muscle atrophy, paralysis, and death. Mitochondrial dysfunction is a major contributor to motor neuron degeneration associated with ALS progression. Mitochondrial abnormalities have been determined in spinal cords of animal disease models and ALS patients. However, molecular mechanisms leading to mitochondrial dysfunction in sporadic ALS (sALS) patients remain unclear...
May 6, 2018: Journal of Neuroscience Research
https://www.readbyqxmd.com/read/29719505/cellular-and-molecular-basis-of-neurodegeneration-in-parkinson-disease
#3
REVIEW
Xian-Si Zeng, Wen-Shuo Geng, Jin-Jing Jia, Lei Chen, Peng-Peng Zhang
It has been 200 years since Parkinson disease (PD) was described by Dr. Parkinson in 1817. The disease is the second most common neurodegenerative disease characterized by a progressive loss of dopaminergic neurons in the substantia nigra pars compacta. Although the pathogenesis of PD is still unknown, the research findings from scientists are conducive to understand the pathological mechanisms. It is well accepted that both genetic and environmental factors contribute to the onset of PD. In this review, we summarize the mutations of main seven genes (α-synuclein, LRRK2, PINK1, Parkin, DJ-1, VPS35 and GBA1) linked to PD, discuss the potential mechanisms for the loss of dopaminergic neurons (dopamine metabolism, mitochondrial dysfunction, endoplasmic reticulum stress, impaired autophagy, and deregulation of immunity) in PD, and expect the development direction for treatment of PD...
2018: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/29704317/development-of-gmp-1-a-molecular-chaperone-network-modulator-protecting-mitochondrial-function-and-its-assessment-in-fly-and-mice-models-of-alzheimer-s-disease
#4
Pavel F Pavlov, Birgit Hutter-Paier, Daniel Havas, Manfred Windisch, Bengt Winblad
Mitochondrial dysfunction is an early feature of Alzheimer's disease (AD) and may play an important role in the pathogenesis of disease. It has been shown that amyloid beta peptide (Aβ) and amyloid precursor protein (APP) interact with mitochondria contributing to the mitochondrial dysfunction in AD. Prevention of abnormal protein targeting to mitochondria can protect normal mitochondrial function, increase neuronal survival and at the end, ameliorate symptoms of AD and other neurodegenerative disorders. First steps of mitochondrial protein import are coordinated by molecular chaperones Hsp70 and Hsp90 that bind to the newly synthesized mitochondria-destined proteins and deliver them to the protein import receptors on the surface of organelle...
April 27, 2018: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/29694829/mitochondrial-camp-pka-signaling-what-do-we-really-know
#5
REVIEW
Yasmine Ould Amer, Etienne Hebert-Chatelain
Mitochondria are key organelles for cellular homeostasis. They generate the most part of ATP that is used by cells through oxidative phosphorylation. They also produce reactive oxygen species, neurotransmitters and other signaling molecules. They are important for calcium homeostasis and apoptosis. Considering the role of this organelle, it is not surprising that most mitochondrial dysfunctions are linked to the development of pathologies. Various mechanisms adjust mitochondrial activity according to physiological needs...
April 22, 2018: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29692267/oxidative-stress-in-neurodegenerative-diseases-a-review
#6
Amirhossein Sahebkar, Yunes Panahi, Habib Yaribeygi, Behjat Javadi
BACKGROUND: Neurodegeneration is a condition in which progressive loss of function and structure of neurons happen. Many lines of evidence suggest that oxidative stress have a central role in neurodegenerative diseases. OBJECTIVE: To survey molecular mechanisms underlying the involvement of oxidative stress in developing different neurodegenerative diseases. METHODS: Original and review articles were retrieved through a PubMed and Google scholar search (from 1989 to 2015) using the following key words: "oxidative stress", "nerve degeneration" and "neurodegenerative diseases"...
April 25, 2018: CNS & Neurological Disorders Drug Targets
https://www.readbyqxmd.com/read/29680477/yap1-tead1-signaling-controls-angiogenesis-and-mitochondrial-biogenesis-through-pgc1%C3%AE
#7
Akiko Mammoto, Megan Muyleart, Andrew Kadlec, David Gutterman, Tadanori Mammoto
Mitochondria contribute to key processes of cellular function, while mitochondrial dysfunction is implicated in metabolic disorders, neurodegenerative diseases, and cardiovascular diseases, in which angiogenesis - the formation of new blood capillaries - is dysregulated. The Hippo signaling transducer, Yes-associated protein (YAP1) binds to the TEA domain (TEAD1) transcription factor and controls angiogenesis. YAP1 also regulates glucose metabolism through peroxisome proliferator-activated receptor gamma co-activator 1-alpha (PGC1α), a major player controlling mitochondrial biogenesis...
April 19, 2018: Microvascular Research
https://www.readbyqxmd.com/read/29677102/implications-of-pi3k-akt-pten-signaling-on-superoxide-dismutases-expression-and-in-the-pathogenesis-of-alzheimer-s-disease
#8
REVIEW
Satoru Matsuda, Yukie Nakagawa, Ai Tsuji, Yasuko Kitagishi, Atsuko Nakanishi, Toshiyuki Murai
Alzheimer’s disease is a neurodegenerative sickness, where the speed of personal disease progression differs prominently due to genetic and environmental factors such as life style. Alzheimer’s disease is described by the construction of neuronal plaques and neurofibrillary tangles composed of phosphorylated tau protein. Mitochondrial dysfunction may be a noticeable feature of Alzheimer’s disease and increased production of reactive oxygen species has long been described. Superoxide dismutases (SODs) protect from excess reactive oxygen species to form less reactive hydrogen peroxide...
April 20, 2018: Diseases (Basel)
https://www.readbyqxmd.com/read/29676481/high-mobility-group-box-1-in-parkinson-s-disease-from-pathogenesis-to-therapeutic-approaches
#9
REVIEW
Efthalia Angelopoulou, Christina Piperi, Athanasios G Papavassiliou
Parkinson's disease (PD) presents the second most common neurodegenerative disorder with largely unknown pathogenesis and inefficient therapeutic management. Accumulating data indicate that neuroinflammation, autophagy impairment, α-synuclein aggregation and mitochondrial dysfunction may contribute to PD onset; however the molecular mechanisms underlying these pathophysiological processes are still under elucidation. Interestingly, recent evidence has indicated that High-mobility group box 1 (HMGB1), a DNA-binding protein that can be actively secreted by inflammatory cells and passively released by necrotic cells may play a key role in PD pathogenesis...
April 20, 2018: Journal of Neurochemistry
https://www.readbyqxmd.com/read/29671352/huntington-s-disease-novel-therapeutic-perspectives-hanging-in-the-balance
#10
Ana Saavedra, Gerardo García-Díaz Barriga, Esther Pérez-Navarro, Jordi Alberch
Huntington's disease (HD), an autosomal dominant neurodegenerative disorder caused by an expansion of CAG repeats in the huntingtin gene, has long been characterized by the presence of motor symptoms due to the loss of striatal projection neurons. Cognitive dysfunction and neuropsychiatric symptoms are also present and they occur in the absence of cell death in most mouse models, pointing to neuronal dysfunction and abnormal synaptic plasticity as causative mechanisms. Areas covered: Here, we focus on those common mechanisms altered by the presence of mutant huntingtin affecting corticostriatal and hippocampal function as therapeutic targets that could prove beneficial to ameliorate both cognitive and motor function in HD...
April 19, 2018: Expert Opinion on Therapeutic Targets
https://www.readbyqxmd.com/read/29656361/characterization-of-the-mitochondrial-aerobic-metabolism-in-the-pre-and-perisynaptic-districts-of-the-sod1-g93a-mouse-model-of-amyotrophic-lateral-sclerosis
#11
Silvia Ravera, Tiziana Bonifacino, Martina Bartolucci, Marco Milanese, Elena Gallia, Francesca Provenzano, Katia Cortese, Isabella Panfoli, Giambattista Bonanno
Amyotrophic lateral sclerosis (ALS) is an adult-onset fatal neurodegenerative disease characterized by muscle wasting, weakness, and spasticity due to a progressive degeneration of cortical, brainstem, and spinal motor neurons. The etiopathological causes are still largely obscure, although astrocytes definitely play a role in neuronal damage. Several mechanisms have been proposed to concur to neurodegeneration in ALS, including mitochondrial dysfunction. We have previously shown profound modifications of glutamate release and presynaptic plasticity in the spinal cord of the SOD1 G93A mouse model of ALS...
April 14, 2018: Molecular Neurobiology
https://www.readbyqxmd.com/read/29653138/neuroprotective-action-of-4-hydroxyisophthalic-acid-against-paraquat-induced-motor-impairment-involves-amelioration-of-mitochondrial-damage-and-neurodegeneration-in-drosophila
#12
Niveditha S, T Shivanandappa
Neurodegenerative disorders including Parkinson's disease (PD) are believed to be caused by oxidative stress and mitochondrial dysfunction. Exposure to environmental agents such as pesticides has been implicated in the etiology of sporadic PD. Paraquat (PQ), a widely used herbicide, induces PD symptoms in laboratory animals including Drosophila. PQ acts as a free radical generator and induces oxidative damage, which is implicated in neuronal cell death. Drosophila model of PQ-induced PD offers a convenient tool for mechanistic studies and, to assess the neuroprotective potential of natural antioxidants...
April 10, 2018: Neurotoxicology
https://www.readbyqxmd.com/read/29645350/degradation-of-altered-mitochondria-by-autophagy-is-impaired-in-lafora-disease
#13
Marcos Lahuerta, Carmen Aguado, Pablo Sánchez-Martín, Pascual Sanz, Erwin Knecht
Lafora disease (LD) is a fatal neurodegenerative disorder caused mostly by mutations in either of two genes encoding laforin and malin. LD is characterized by accumulation of a poorly-branched form of glycogen in the cytoplasm of neurons and other cells. We previously reported dysfunctional mitochondria in different LD models. Now, using mitochondrial uncouplers and respiratory chain inhibitors, we have investigated with human fibroblasts a possible alteration in the selective degradation of damaged mitochondria (mitophagy) in LD...
April 12, 2018: FEBS Journal
https://www.readbyqxmd.com/read/29644751/disease-modification-in-parkinson-s-disease-current-approaches-challenges-and-future-considerations
#14
REVIEW
Anthony E Lang, Alberto J Espay
The greatest unmet therapeutic need in Parkinson's disease is the development of treatment that slows the relentless progression of the neurodegenerative process. The concept of "disease modification" encompasses intervention types ranging from those designed to slow the underlying degeneration to treatments directed at regenerating or replacing lost neurons. To date all attempts to develop effective disease-modifying therapy have failed. Many reasons have been proposed for these failures including our rudimentary understanding of disease pathogenesis and the assumption that each targeted mechanisms of disease apply to most patients with the same clinical diagnosis...
April 11, 2018: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/29625119/the-exosome-of-adipose-derived-stem-cells-reduces-%C3%AE-amyloid-pathology-and-apoptosis-of-neuronal-cells-derived-from-the-transgenic-mouse-model-of-alzheimer-s-disease
#15
Mijung Lee, Jae-Jun Ban, Seungwon Yang, Wooseok Im, Manho Kim
Adipose-derived stem cells (ADSC) have a therapeutic potential for the treatment of neurodegenerative disorders such as Alzheimer's disease (AD). Exosomes are extracellular vesicles secreted from various types of cells, and stem cell-derived exosomes are known to have beneficial effects in many diseases. Many studies have suggested that amyloid beta (Aβ) peptides have a pivotal role in AD progression, by mitochondrial dysfunction of neuronal cells. We examined the therapeutic potential of exosomes derived from ADSCs (ADSC-Exo) in preventing the disease phenotypes induced by the Aβ cascade in an AD in vitro model...
April 3, 2018: Brain Research
https://www.readbyqxmd.com/read/29624777/de-novo-tetrahydrobiopterin-biosynthesis-is-impaired-in-the-inflammed-striatum-of-parkin-mice
#16
Roberta de Paula Martins, Viviane Glaser, Aderbal S Aguiar, Priscila Maximiliano de Paula Ferreira, Karina Ghisoni, Débora da Luz Scheffer, Laurence Lanfumey, Rita Raisman-Vozari, Olga Corti, Ana Lucia De Paul, Rodrigo Augusto da Silva, Alexandra Latini
Parkinson's disease (PD), the second-most prevalent neurodegenerative disease, is primarily characterized by neurodegeneration in the substantia nigra pars compacta, resulting in motor impairment. Loss-of-function mutations in parkin are the major cause of the early-onset familial form of the disease. Although rodents deficient in parkin (parkin(-/-)) have some dopaminergic system dysfunction associated with central oxidative stress and energy metabolism deficiencies, these animals only display nigrostriatal pathway degeneration under inflammatory conditions...
April 6, 2018: Cell Biology International
https://www.readbyqxmd.com/read/29624358/toxicity-of-beta-amyloid-protein-during-aging-influence-of-synaptosomal-energy-metabolism
#17
(no author information available yet)
Background: Recent evidence suggests that early neurodegenerative events associated with Alzheimer’s disease (AD) probably begin in the synaptic terminal, where it has been reported a large accumulation of β-amyloid protein (Aβ), one of the main factors described in the development of AD. We analyzed the influence of energy metabolism on the toxic effects of Aβ during aging on synaptosomes from neocortex and hippocampus of rats exposed to inhibitors of glycolytic and mitochondrial metabolism and we evaluated the protective effects of some antioxidant compounds...
2018: Revista Médica del Instituto Mexicano del Seguro Social
https://www.readbyqxmd.com/read/29619740/nicotine-modulates-mitochondrial-dynamics-in-hippocampal-neurons
#18
Juan A Godoy, Angel G Valdivieso, Nibaldo C Inestrosa
Mitochondria are widely recognized as fundamental organelles for cellular physiology and constitute the main energy source for different cellular processes. The location, morphology, and interactions of mitochondria with other organelles, such as the endoplasmic reticulum (ER), have emerged as critical events capable of determining cellular fate. Mitochondria-related functions have proven particularly relevant in neurons; mitochondria are necessary for proper neuronal morphogenesis and the highly energy-demanding synaptic transmission process...
April 4, 2018: Molecular Neurobiology
https://www.readbyqxmd.com/read/29616350/mitochondrial-dysfunction-in-parkinson-s-disease-new-mechanistic-insights-and-therapeutic-perspectives
#19
REVIEW
Jin-Sung Park, Ryan L Davis, Carolyn M Sue
PURPOSE OF REVIEW: Parkinson's disease (PD) is a complex neurodegenerative disorder, the aetiology of which is still largely unknown. Overwhelming evidence indicates that mitochondrial dysfunction is a central factor in PD pathophysiology. Here we review recent developments around mitochondrial dysfunction in familial and sporadic PD, with a brief overview of emerging therapies targeting mitochondrial dysfunction. RECENT FINDINGS: Increasing evidence supports the critical role for mitochondrial dysfunction in the development of sporadic PD, while the involvement of familial PD-related genes in the regulation of mitochondrial biology has been expanded by the discovery of new mitochondria-associated disease loci and the identification of their novel functions...
April 3, 2018: Current Neurology and Neuroscience Reports
https://www.readbyqxmd.com/read/29605485/a-camp-analog-attenuates-beta-amyloid-1-42-induced-mitochondrial-dysfunction-and-spatial-learning-and-memory-deficits
#20
Mehdi Aghsami, Mohammad Sharifzadeh, Mohammad Reza Sepand, Meysam Yazdankhah, Seyed Afshin Seyednejad, Jalal Pourahmad
Alzheimer's disease (AD), a neurodegenerative disorder in elderly, is indicated with deposition of Amyloid β (Aβ) in the brain and accompanied with cognitive impairment. Bucladesine, a phosphodiesterase inhibitor, may ameliorate AD's cognitive dysfunctions through mimicking the action of cAMP and raising its intracellular level. Here, we investigated the effects of bucladesine on Aβ-induced memory and learning impairment in a Morris water maze (MWM) model. Rats were injected with bucladesine (1 μl/side from a 100 μM stock solution) and Aβ (1 μl/side from a 100 μM stock solution) intra-hippocampally and after 19 days were trained for 4 successive days...
March 29, 2018: Brain Research Bulletin
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"