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https://www.readbyqxmd.com/read/29053833/clinical-pathological-and-functional-characterization-of-riboflavin-responsive-neuropathy
#1
Andreea Manole, Zane Jaunmuktane, Iain Hargreaves, Marthe H R Ludtmann, Vincenzo Salpietro, Oscar D Bello, Simon Pope, Amelie Pandraud, Alejandro Horga, Renata S Scalco, Abi Li, Balasubramaniem Ashokkumar, Charles M Lourenço, Simon Heales, Rita Horvath, Patrick F Chinnery, Camilo Toro, Andrew B Singleton, Thomas S Jacques, Andrey Y Abramov, Francesco Muntoni, Michael G Hanna, Mary M Reilly, Tamas Revesz, Dimitri M Kullmann, James E C Jepson, Henry Houlden
Brown-Vialetto-Van Laere syndrome represents a phenotypic spectrum of motor, sensory, and cranial nerve neuropathy, often with ataxia, optic atrophy and respiratory problems leading to ventilator-dependence. Loss-of-function mutations in two riboflavin transporter genes, SLC52A2 and SLC52A3, have recently been linked to Brown-Vialetto-Van Laere syndrome. However, the genetic frequency, neuropathology and downstream consequences of riboflavin transporter mutations are unclear. By screening a large cohort of 132 patients with early-onset severe sensory, motor and cranial nerve neuropathy we confirmed the strong genetic link between riboflavin transporter mutations and Brown-Vialetto-Van Laere syndrome, identifying 22 pathogenic mutations in SLC52A2 and SLC52A3, 14 of which were novel...
September 26, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29053743/in-vitro-modeling-of-experimental-succinic-semialdehyde-dehydrogenase-deficiency-ssadhd-using-brain-derived-neural-stem-cells
#2
Kara R Vogel, Garrett R Ainslie, Erwin E Jansen, Gajja S Salomons, Jean-Baptiste Roullet, K Michael Gibson
We explored the utility of neural stem cells (NSCs) as an in vitro model for evaluating preclinical therapeutics in succinic semialdehyde dehydrogenase-deficient (SSADHD) mice. NSCs were obtained from aldh5a1+/+ and aldh5a1-/- mice (aldh5a1 = aldehyde dehydrogenase 5a1 = SSADH). Multiple parameters were evaluated including: (1) production of GHB (γ-hydroxybutyrate), the biochemical hallmark of SSADHD; (2) rescue from cell death with the dual mTOR (mechanistic target of rapamycin) inhibitor, XL-765, an agent previously shown to rescue aldh5a1-/- mice from premature lethality; (3) mitochondrial number, total reactive oxygen species, and mitochondrial superoxide production, all previously documented as abnormal in aldh5a1-/- mice; (4) total ATP levels and ATP consumption; and (5) selected gene expression profiles associated with epilepsy, a prominent feature in both experimental and human SSADHD...
2017: PloS One
https://www.readbyqxmd.com/read/29050859/neuroprotective-effects-of-a-triple-glp-1-gip-glucagon-receptor-agonist-in-the-app-ps1-transgenic-mouse-model-of-alzheimer-s-disease
#3
Jingjing Tai, Weizhen Liu, Yanwei Li, Lin Li, Christian Hölscher
Type 2 diabetes mellitus (T2DM) is a risk factor for Alzheimer disease (AD). Previous studies have shown that the incretin hormones glucagon-like peptide-1 (GLP-1) and glucose-dependent insulinotropic polypeptide (GIP) that have anti-diabetic properties show very promising effects in animal models of AD. Glucagon (Gcg) is a hormone and growth-factor, and the Gcg receptor is expressed in the brain. Here we test the effects of a triple receptor agonist (TA), which activates GIP-1, GIP and glucagon receptors at the same time...
October 16, 2017: Brain Research
https://www.readbyqxmd.com/read/29050512/iron-deficiency-beyond-erythropoiesis-should-we-be-concerned
#4
Khaled M Musallam, Ali T Taher
OBJECTIVE: To consider the key implications of iron deficiency for biochemical and physiological functions beyond erythropoiesis. METHODS: PubMed was searched for relevant journal articles published up to August 2017. RESULTS: Anemia is the most well-recognized consequence of persisting iron deficiency, but various other unfavorable consequences can develop either before or concurrently with anemia. Mitochondrial function can be profoundly disturbed since iron is a cofactor for heme-containing enzymes and non-heme iron-containing enzymes in the mitochondrial electron transport chain...
October 20, 2017: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/29049383/lutein-accumulates-in-subcellular-membranes-of-brain-regions-in-adult-rhesus-macaques-relationship-to-dha-oxidation-products
#5
Emily S Mohn, John W Erdman, Matthew J Kuchan, Martha Neuringer, Elizabeth J Johnson
OBJECTIVES: Lutein, a carotenoid with anti-oxidant functions, preferentially accumulates in primate brain and is positively related to cognition in humans. Docosahexaenoic acid (DHA), an omega-3 polyunsaturated fatty acid (PUFA), is also beneficial for cognition, but is susceptible to oxidation. The present study characterized the membrane distribution of lutein in brain regions important for different domains of cognitive function and determined whether membrane lutein was associated with brain PUFA oxidation...
2017: PloS One
https://www.readbyqxmd.com/read/29047383/a-novel-small-molecule-inhibitor-of-p32-mitochondrial-protein-overexpressed-in-glioma
#6
Venkata Yenugonda, Natsuko Nomura, Valentina Kouznetsova, Igor Tsigelny, Valentina Fogal, Elmar Nurmemmedov, Santosh Kesari, Ivan Babic
BACKGROUND: The mitochondrial protein p32 is a validated therapeutic target of cancer overexpressed in glioma. Therapeutic targeting of p32 with monoclonal antibody or p32-binding LyP-1 tumor-homing peptide can limit tumor growth. However, these agents do not specifically target mitochondrial-localized p32 and would not readily cross the blood-brain barrier to target p32-overexpressing gliomas. Identifying small molecule inhibitors of p32 overexpressed in cancer is a more rational therapeutic strategy...
October 18, 2017: Journal of Translational Medicine
https://www.readbyqxmd.com/read/29046694/hyperglycemia-exacerbates-downregulation-of-dynamin-like-protein-1-in-ischemic-cerebral-injury
#7
Dong-Ju Park, Myeong-Ok Kim, Phil-Ok Koh
Ischemic stroke is one of the leading causes of adult disability and death. Hyperglycemia is associated with an increased risk of stroke and poor outcomes after brain injury. Dynamin-like protein I (DLP-1) regulates mitochondrial fission and promotes mitochondrial dynamics. Neurodegenerative diseases are associated with mitochondrial dysfunction, and the downregulation of DLP-1 has been previously identified in a stroke animal model. Here, we investigated the changes in DLP-1 protein expression in an animal model of focal cerebral ischemia with induced hyperglycemia...
September 2017: Laboratory Animal Research
https://www.readbyqxmd.com/read/29045576/activation-of-cannabinoid-receptor-1-is-involved-in-protection-against-mitochondrial-dysfunction-and-cerebral-ischaemic-tolerance-induced-by-isoflurane-preconditioning
#8
M Cai, Q Yang, G Li, S Sun, Y Chen, L Tian, H Dong
Background: Isoflurane preconditioning (IPC) induces cerebral ischaemic tolerance, but the mechanism remains poorly understood. The aim of this study was to determine changes in mitochondrial function in the brain after IPC, and whether the cannabinoid receptor 1 (CB1R) could be involved in the mechanism of mitochondrial protection mediated by IPC. Methods: Adult male Sprague-Dawley rats were pretreated with isoflurane 2% for 1 h day-1, for 5 days consecutively, and then subjected to 120 min right middle cerebral artery occlusion...
October 13, 2017: British Journal of Anaesthesia
https://www.readbyqxmd.com/read/29045486/thiamine-deficiency-activates-hypoxia-inducible-factor-1%C3%AE-to-facilitate-pro-apoptotic-responses-in-mouse-primary-astrocytes
#9
Kristy Zera, Jason Zastre
Thiamine is an essential enzyme cofactor required for proper metabolic function and maintenance of metabolism and energy production in the brain. In developed countries, thiamine deficiency (TD) is most often manifested following chronic alcohol consumption leading to impaired mitochondrial function, oxidative stress, inflammation and excitotoxicity. These biochemical lesions result in apoptotic cell death in both neurons and astrocytes. Comparable histological injuries in patients with hypoxia/ischemia and TD have been described in the thalamus and mammillary bodies, suggesting a congruency between the cellular responses to these stresses...
2017: PloS One
https://www.readbyqxmd.com/read/29043143/focal-segmental-glomerulosclerosis-associated-with-mitochondrial-disease
#10
Kenneth Lim, David Steele, Andrew Fenves, Ravi Thadhani, Eliot Heher, Amel Karaa
Primary mitochondrial diseases (MD) are complex, heterogeneous inherited diseases caused by mutations in either the mitochondrial or nuclear DNA. Glomerular diseases in MD have been reported with tRNA mutation m.3243A>G causing a syndrome of mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS). We describe here a case of focal segmental glomerulosclerosis (FSGS) associated with a new tRNA mutation site. A 34-year-old man with a history of living related kidney transplantation, diabetes, hearing loss, and developmental delay presented to the outpatient clinic with complaints of new behavioral difficulties, worsening symptoms, and brain involvement on imaging...
2017: Clin Nephrol Case Stud
https://www.readbyqxmd.com/read/29042253/calcium-uptake-and-cytochrome-c-release-from-normal-and-ischemic-brain-mitochondria
#11
Alexander Andreyev, Pratistha Tamrakar, Robert E Rosenthal, Gary Fiskum
At abnormally elevated levels of intracellular Ca(2+), mitochondrial Ca(2+) uptake may compromise mitochondrial electron transport activities and trigger membrane permeability changes that allow for release of cytochrome c and other mitochondrial apoptotic proteins into the cytosol. In this study, a clinically relevant canine cardiac arrest model was used to assess the effects of global cerebral ischemia and reperfusion on mitochondrial Ca(2+) uptake capacity, Ca(2+) uptake-mediated inhibition of respiration, and Ca(2+)-induced cytochrome c release, as measured in vitro in a K(+)-based medium in the presence of Mg(2+), ATP, and NADH-linked oxidizable substrates...
October 14, 2017: Neurochemistry International
https://www.readbyqxmd.com/read/29042133/determination-of-mitochondrial-metabolic-phenotype-through-investigation-of-the-intrinsic-inhibition-of-succinate-dehydrogenase
#12
Alexander Panov, Zulfiya Orynbayeva
Many diseases are accompanied by systemic or organ metabolic abnormalities. Therefore, investigation of the roles of mitochondrial dysfunction in the pathogenesis of major diseases requires a methodology that reflects the characteristics of mitochondrial metabolism particular for the organ of origin. We provide evidence that for brain and heart mitochondria the intrinsic inhibition of succinate dehydrogenase (SDH) is a key mechanism for attenuation of mitochondrial respiration and energy production in response to the organ's energy needs...
October 14, 2017: Analytical Biochemistry
https://www.readbyqxmd.com/read/29040828/neuropsychological-functioning-and-brain-energetics-of-drug-resistant-mesial-temporal-lobe-epilepsy-patients
#13
Camila Moreira Osório, Alexandra Latini, Rodrigo Bainy Leal, Maria Emília Rodrigues de Oliveira Thais, Helena Dresch Vascouto, Aline Pertile Remor, Mark William Lopes, Marcelo Neves Linhares, Juliana Ben, Roberta de Paula Martins, Rui Daniel Prediger, Alexandre Ademar Hoeller, Hans Joachim Markowitsch, Peter Wolf, Kátia Lin, Roger Walz
Interictal hypometabolism is commonly measured by 18-fluoro-deoxyglucose Positron Emission Tomography (FDG-PET) in the temporal lobe of patients with mesial temporal lobe epilepsy (MTLE-HS). Left temporal lobe interictal FDG-PET hypometabolism has been associated with verbal memory impairment, while right temporal lobe FDG-PET hypometabolism is associated with nonverbal memory impairment. The biochemical mechanisms involved in these findings remain unknown. In comparison to healthy controls (n=21), surgically treated patients with MTLE-HS (n=32, left side=17) had significant lower scores in the Rey Auditory Verbal Learning Test (RAVLT retention and delayed), Logical Memory II (LMII), Boston Naming test (BNT), Letter Fluency and Category Fluency...
October 12, 2017: Epilepsy Research
https://www.readbyqxmd.com/read/29039658/membrane-binding-and-pore-formation-by-a-cytotoxic-fragment-of-amyloid-%C3%AE-peptide
#14
Nabin Kandel, Tianyu Zheng, Qun Huo, Suren A Tatulian
Amyloid β (Aβ) peptide contributes to Alzheimer's disease by a yet unidentified mechanism. In brain tissue, Aβ occurs in various forms, including an undecapeptide Aβ25-35, which exerts neurotoxic effect through mitochondrial dysfunction and/or Ca(2+)-permeable pore formation in cell membranes. This work was aimed at biophysical characterization of membrane binding and pore formation by Aβ25-35. Interaction of Aβ25-35 with anionic and zwitterionic membranes was analyzed by microelectrophoresis. In pore formation experiments, Aβ25-35 was incubated in aqueous buffer to form oligomers and added to Quin-2-loaded vesicles...
October 17, 2017: Journal of Physical Chemistry. B
https://www.readbyqxmd.com/read/29038583/zdhhc13-dependent-drp1-s-palmitoylation-impacts-brain-bioenergetics-anxiety-coordination-and-motor-skills
#15
Eleonora Napoli, Gyu Song, Siming Liu, Alexsandra Espejo, Carlos J Perez, Fernando Benavides, Cecilia Giulivi
Protein S-palmitoylation is a reversible post-translational modification mediated by palmitoyl acyltransferase enzymes, a group of Zn(2+)-finger DHHC-domain-containing proteins (ZDHHC). Here, for the first time, we show that Zdhhc13 plays a key role in anxiety-related behaviors and motor function, as well as brain bioenergetics, in a mouse model (luc) carrying a spontaneous Zdhhc13 recessive mutation. At 3 m of age, mutant mice displayed increased sensorimotor gating, anxiety, hypoactivity, and decreased motor coordination, compared to littermate controls...
October 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29038245/mitochondrial-calcium-dysregulation-contributes-to-dendrite-degeneration-mediated-by-pd-lbd-associated-lrrk2-mutants
#16
Manish Verma, Jason Callio, P Anthony Otero, Israel Sekler, Zachary P Wills
Mutations in leucine-rich repeat kinase 2 (LRRK2) contribute to development of late-onset familial Parkinson's disease (PD), with clinical features of motor and cognitive dysfunction indistinguishable from sporadic PD. Calcium dysregulation plays an important role in PD pathogenesis, but the mechanisms of neurodegeneration remain unclear. Recent reports indicate enhanced excitatory neurotransmission in cortical neurons expressing mutant LRRK2, which occurs prior to the well-characterized phenotype of dendritic shortening...
October 16, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/29034965/role-of-serum-albumin-as-a-nanoparticulate-carrier-for-nose-to-brain-delivery-of-r-flurbiprofen-implications-for-the-treatment-of-alzheimer-s-disease
#17
Ling Rong Wong, Paul C Ho
OBJECTIVES: R-flurbiprofen (R-FP) was found to offer neuroprotective effects by inhibiting mitochondrial calcium overload induced by β-amyloid peptide toxicity in Alzheimer's disease (AD). However, poor brain penetration after oral administration posed a challenge to its further development for AD treatment. In this study, we investigated the potential of serum albumin as nanoparticulate carriers for nose-to-brain delivery of R-FP to improve its brain accumulation. METHODS: Mice were subjected to three treatment groups: (1) intranasal R-FP solution, (2) oral R-FP solution and (3) intranasal R-FP albumin nanoparticles...
October 16, 2017: Journal of Pharmacy and Pharmacology
https://www.readbyqxmd.com/read/29034461/the-migraine-attack-as-a-homeostatic-neuroprotective-response-to-brain-oxidative-stress-preliminary-evidence-for-a-theory
#18
Jonathan M Borkum
BACKGROUND: Previous research has suggested that migraineurs show higher levels of oxidative stress (lipid peroxides) between migraine attacks and that migraine triggers may further increase brain oxidative stress. Oxidative stress is transduced into a neural signal by the TRPA1 ion channel on meningeal pain receptors, eliciting neurogenic inflammation, a key event in migraine. Thus, migraines may be a response to brain oxidative stress. RESULTS: In this article, a number of migraine components are considered: cortical spreading depression, platelet activation, plasma protein extravasation, endothelial nitric oxide synthesis, and the release of serotonin, substance P, calcitonin gene-related peptide, and brain-derived neurotrophic factor...
October 16, 2017: Headache
https://www.readbyqxmd.com/read/29031832/initial-brain-aging-heterogeneity-of-mitochondrial-size-is-associated-with-decline-in-complex-i-linked-respiration-in-cortex-and-hippocampus
#19
Kirsten Thomsen, Takashi Yokota, Md Mahdi Hasan-Olive, Niloofar Sherazi, Nima Borhan Fakouri, Claus Desler, Christine Elisabeth Regnell, Steen Larsen, Lene Juel Rasmussen, Flemming Dela, Linda Hildegard Bergersen, Martin Lauritzen
Brain aging is accompanied by declining mitochondrial respiration. We hypothesized that mitochondrial morphology and dynamics would reflect this decline. Using hippocampus and frontal cortex of a segmental progeroid mouse model lacking Cockayne syndrome protein B (CSB(m/m)) and C57Bl/6 (WT) controls and comparing young (2-5 months) to middle-aged mice (13-14 months), we found that complex I-linked state 3 respiration (CI) was reduced at middle age in CSB(m/m) hippocampus, but not in CSB(m/m) cortex or WT brain...
August 12, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/29031712/mitochondrial-uncoupling-in-the-melanocortin-system-differentially-regulates-npy-and-pomc-neurons-to-promote-weight-loss
#20
Natalie Jane Michael, Stephanie Elise Simonds, Marco van den Top, Michael Alexander Cowley, David Spanswick
OBJECTIVE: The mitochondrial uncoupling agent 2,4-dinitrophenol (DNP), historically used as a treatment for obesity, is known to cross the blood-brain-barrier, but its effects on central neural circuits controlling body weight are largely unknown. As hypothalamic melanocortin neuropeptide Y/agouti-related protein (NPY/AgRP) and pro-opiomelanocortin (POMC) neurons represent key central regulators of food intake and energy expenditure we investigated the effects of DNP on these neurons, food intake and energy expenditure...
October 2017: Molecular Metabolism
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