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https://www.readbyqxmd.com/read/29454001/brain-bioenergetics-in-rats-with-acute-hyperphenylalaninemia
#1
Nádia Weber Dimer, Bruna Klippel Ferreira, Jotele Fontana Agostini, Maria Luiza Gomes, Luiza Wilges Kist, Fernanda Malgarin, Milena Carvalho-Silva, Lara Mezari Gomes, Joyce Rebelo, Marisa Jádna Silva Frederico, Fátima Regina Mena Barreto Silva, Eduardo Pacheco Rico, Mauricio Reis Bogo, Emilio Luiz Streck, Gustavo Costa Ferreira, Patrícia Fernanda Schuck
Phenylketonuria (PKU) is a disorder of phenylalanine (Phe) metabolism caused by deficient phenylalanine hydroxylase activity. The deficiency results in increased levels of Phe and its metabolites in fluids and tissues of patients. PKU patients present neurological signals and symptoms including hypomyelination and intellectual deficit. This study assessed brain bioenergetics at 1 h after acute Phe administration to induce hyperphenylalaninemia (HPA) in rats. Wistar rats were randomized in two groups: HPA animals received a single subcutaneous administration of Phe (5...
February 14, 2018: Neurochemistry International
https://www.readbyqxmd.com/read/29448100/post-ischemic-administration-of-5-methoxyindole-2-carboxylic-acid-at-the-onset-of-reperfusion-affords-neuroprotection-against-stroke-injury-by-preserving-mitochondrial-function-and-attenuating-oxidative-stress
#2
Jinzi Wu, Zhen Jin, Xiaorong Yang, Liang-Jun Yan
We previously reported that 5-methoxyindole-2-carboxylic acid (MICA) could induce preconditioning effect in the ischemic brain of rat. In the present study, we addressed the question of whether MICA could also trigger a postconditioning effect in ischemic stroke. To this end, MICA (100mgkg body weight) was injected intraperitoneally at the onset of 24 h reperfusion following 1 h ischemia in rat brain. Results indicate that stroked animals treated with MICA showed less brain infarction volume than that of vehicle-treated animals...
February 12, 2018: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/29447265/dynamic-remodeling-of-lipids-coincides-with-dengue-virus-replication-in-the-midgut-of-aedes-aegypti-mosquitoes
#3
Nunya Chotiwan, Barbara G Andre, Irma Sanchez-Vargas, M Nurul Islam, Jeffrey M Grabowski, Amber Hopf-Jannasch, Erik Gough, Ernesto Nakayasu, Carol D Blair, John T Belisle, Catherine A Hill, Richard J Kuhn, Rushika Perera
We describe the first comprehensive analysis of the midgut metabolome of Aedes aegypti, the primary mosquito vector for arboviruses such as dengue, Zika, chikungunya and yellow fever viruses. Transmission of these viruses depends on their ability to infect, replicate and disseminate from several tissues in the mosquito vector. The metabolic environments within these tissues play crucial roles in these processes. Since these viruses are enveloped, viral replication, assembly and release occur on cellular membranes primed through the manipulation of host metabolism...
February 2018: PLoS Pathogens
https://www.readbyqxmd.com/read/29447225/low-apolipoprotein-a-i-levels-in-friedreich-s-ataxia-and-in-frataxin-deficient-cells-implications-for-therapy
#4
QingQing Wang, Lili Guo, Cassandra J Strawser, Lauren A Hauser, Wei-Ting Hwang, Nathaniel W Snyder, David R Lynch, Clementina Mesaros, Ian A Blair
Friedreich's ataxia (FA) is an autosomal recessive neurodegenerative disorder, which results primarily from reduced expression of the mitochondrial protein frataxin. FA has an estimated prevalence of one in 50,000 in the population, making it the most common hereditary ataxia. Paradoxically, mortality arises most frequently from cardiomyopathy and cardiac failure rather than from neurological effects. Decreased high-density lipoprotein (HDL) and apolipoprotein A-I (ApoA-l) levels in the general population are associated with an increased risk of mortality from cardiomyopathy and heart failure...
2018: PloS One
https://www.readbyqxmd.com/read/29436584/mitochondrial-protective-mechanism-of-simvastatin-protects-against-amyloid%C3%A2-%C3%AE-peptide-induced-injury-in-sh-sy5y-cells
#5
Yunzi Li, Qian Liu, Jing Sun, Jin Wang, Xinfeng Liu, Jing Gao
Mitochondrial dysfunction is implicated in the pathology of neuronal damage during Alzheimer's disease (AD). Previous studies suggest that simvastatin (SV) ameliorates amyloid β (Aβ)‑mediated cognitive impairment in AD patients and transgenic mice; however, the mechanisms remain unknown. To investigate the potential mechanisms by which SV protects against AD neurotoxicity, the present study used a series of cellular and molecular assays to analyze the effects of SV in an in vitro model of Aβ1‑42-induced injury...
February 5, 2018: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/29434802/an-investigation-of-the-mechanism-of-dexmedetomidine-in-improving-postoperative-cognitive-dysfunction-from-the-perspectives-of-alleviating-neuronal-mitochondrial-membrane-oxidative-stress-and-electrophysiological-dysfunction
#6
Jie Chen, Na Shen, Xiaohui Duan, Yaning Guo
The aim of this study was to investigate the mechanism of dexmedetomidine in improving postoperative cognitive dysfunction from the perspectives of alleviating neuronal mitochondrial membrane oxidative stress and electrophysiological dysfunction. A total of 120 patients undergoing elective surgery under general anesthesia from June 2013 to May, 2016 were selected as the subjects of the study and randomly divided into the propofol + remifentanil and dexmedetomidine groups. The Rey Auditory Verbal Learning Test (AVLT) and Beck Depression Inventory (BDI) were performed at day 1 before operation and at day 1, 3, 5 and 15 after operation...
February 2018: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29433569/mh84-improves-mitochondrial-dysfunction-in-a-mouse-model-of-early-alzheimer-s-disease
#7
Maximilian Pohland, Maren Pellowska, Heike Asseburg, Stephanie Hagl, Martina Reutzel, Aljoscha Joppe, Dirk Berressem, Schamim H Eckert, Mario Wurglics, Manfred Schubert-Zsilavecz, Gunter P Eckert
BACKGROUND: Current approved drugs for Alzheimer's disease (AD) only attenuate symptoms, but do not cure the disease. The pirinixic acid derivate MH84 has been characterized as a dual gamma-secretase/proliferator activated receptor gamma (PPARγ) modulator in vitro. Pharmacokinetic studies in mice showed that MH84 is bioavailable after oral administration and reaches the brain. We recently demonstrated that MH84 improved mitochondrial dysfunction in a cellular model of AD. In the present study, we extended the pharmacological characterization of MH84 to 3-month-old Thy-1 AβPPSL mice (harboring the Swedish and London mutation in human amyloid precursor protein (APP)) which are characterized by enhanced AβPP processing and cerebral mitochondrial dysfunction, representing a mouse model of early AD...
February 13, 2018: Alzheimer's Research & Therapy
https://www.readbyqxmd.com/read/29433206/lycopene-rich-extract-from-red-guava-psidium-guajava-l-displays-cytotoxic-effect-against-human-breast-adenocarcinoma-cell-line-mcf-7-via-an-apoptotic-like-pathway
#8
Raimunda C Dos Santos, Alicia S Ombredane, Jéssica Maria T Souza, Andreanne G Vasconcelos, Alexandra Plácido, Adriany das G N Amorim, Eder Alves Barbosa, Filipe C D A Lima, Cristina D Ropke, Michel M M Alves, Daniel D R Arcanjo, Fernando A A Carvalho, Cristina Delerue-Matos, Graziella A Joanitti, José Roberto de S A Leite
This study investigated a lycopene-rich extract from red guava (LEG) for its chemical composition using spectrophotometry, mass spectrometry, attenuated total reflectance-fourier transform infrared spectroscopy (ATR-FTIR), and computational studies. The cytotoxic activity of LEG and the underlying mechanism was studied in human breast adenocarcinoma cells (MCF-7), murine fibroblast cells (NIH-3T3), BALB/c murine peritoneal macrophages, and sheep blood erythrocytes by evaluating the cell viability with 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide (MTT) method and flow cytometry...
March 2018: Food Research International
https://www.readbyqxmd.com/read/29432738/humanin-promotes-mitochondrial-biogenesis-in-pancreatic-min6-%C3%AE-cells
#9
Qingqing Qin, Jieqiong Jin, Fang He, Yongqin Zheng, Tingting Li, Yun Zhang, Jundong He
Mitochondrial dysfunction is associated with β-cell failure and insulin resistance in diabetes. Humanin is an endogenous cytoprotective peptide. In the current study, we aimed to define the effects of Humanin on mitochondrial biogenesis in pancreatic β-cells. Our findings demonstrated that Humanin treatment significantly increased the expression of PGC-1α and its downstream target genes NRF1 and TFAM in MIN6 β-cells. Notably, Humanin treatment significantly promoted mitochondrial biogenesis by increasing mitochondrial mass, elevating mtDNA/nDNA ratio, and stimulating the expression of cytochrome B, which were suppressed by the specific AMPK inhibitor compound C...
February 9, 2018: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/29429536/are-patients-affected-by-mitochondrial-disorders-at-nutritional-risk
#10
Emilie Aubry, Carla Aeberhard, Lia Bally, Jean-Marc Nuoffer, Lorenz Risch, Stefan Mühlebach, Jean-Marc Burgunder, Zeno Stanga
OBJECTIVES: Patients with mitochondrial disorders (MD) frequently present with gastrointestinal complaints, mainly gastrointestinal dysmotility, that interfere with their food intake. A deterioration of their nutritional state may worsen the course of the disease. Our study aimed to evaluate a simple screening tool to identify nutritional risk and perform an extended nutritional assessment to explore the potential presence of deficiencies in this population compared with controls. METHODS: A prospective cohort study was conducted to compare outpatients with MD to matched healthy controls...
March 2018: Nutrition
https://www.readbyqxmd.com/read/29425965/the-small-heat-shock-proteins-especially-hspb4-and-hspb5-are-promising-protectants-in-neurodegenerative-diseases
#11
REVIEW
Zhihui Zhu, Georg Reiser
Small heat shock proteins (sHsps) are a group of proteins with molecular mass between 12 and 43 kDa. Currently, 11 members of this family have been classified, namely HspB1 to HspB11. HspB1, HspB2, HspB5, HspB6, HspB7, and HspB8, which are expressed in brain have been observed to be related to the pathology of neurodegenerative diseases, including Parkinson's, Alzheimer's, Alexander's disease, multiple sclerosis, and human immunodeficiency virus-associated dementia. Specifically, sHsps interact with misfolding and damaging protein aggregates, like Glial fibrillary acidic protein in AxD, β-amyloid peptides aggregates in Alzheimer's disease, Superoxide dismutase 1 in Amyotrophic lateral sclerosis and cytosine-adenine-guanine/polyglutamine (CAG/PolyQ) in Huntington's disease, Spinocerebellar ataxia type 3, Spinal-bulbar muscular atrophy, to reduce the toxicity or increase the clearance of these protein aggregates...
February 6, 2018: Neurochemistry International
https://www.readbyqxmd.com/read/29424033/mitochondrial-dysfunction-in-human-skeletal-muscle-biopsies-of-lipid-storage-disorder
#12
B Debashree, Manish Kumar, T S Keshava Prasad, Archana Natarajan, Rita Christopher, A Nalini, P S Bindu, N Gayathri, M M Srinivas Bharath
Mitochondria regulate the balance between lipid metabolism and storage in the skeletal muscle. Altered lipid transport, metabolism and storage influence the bioenergetics, redox status and insulin signalling contributing to cardiac and neurological diseases. Lipid storage disorders (LSDs) are neurological disorders which entail intramuscular lipid accumulation and impaired mitochondrial bioenergetics in the skeletal muscle causing progressive myopathy with muscle weakness. However, the mitochondrial changes including molecular events associated with impaired lipid storage have not been completely understood in the human skeletal muscle...
February 9, 2018: Journal of Neurochemistry
https://www.readbyqxmd.com/read/29423831/targeted-versus-untargeted-omics-the-cafsa-story
#13
Maria Del Mar Amador, Benoit Colsch, Foudil Lamari, Claude Jardel, Farid Ichou, Agnès Rastetter, Frédéric Sedel, Fabien Jourdan, Clément Frainay, Ronald A Wevers, Emmanuel Roze, Christel Depienne, Christophe Junot, Fanny Mochel
BACKGROUND: In 2009, untargeted metabolomics led to the delineation of a new clinico-biological entity called cerebellar ataxia with elevated cerebrospinal free sialic acid, or CAFSA. In order to elucidate CAFSA, we applied sequentially targeted and untargeted omic approaches. METHODS AND RESULTS: First, we studied five of the six CAFSA patients initially described. Besides increased CSF free sialic acid concentrations, three patients presented with markedly decreased 5-methyltetrahydrofolate (5-MTHF) CSF concentrations...
February 8, 2018: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29423070/salinomycin-inhibits-cholangiocarcinoma-growth-by-inhibition-of-autophagic-flux
#14
Johannes Klose, Engin Guerlevik, Tina Trostel, Florian Kühnel, Thomas Schmidt, Martin Schneider, Alexis Ulrich
Introduction: Cholangiocarcinoma is characterized by aggressive tumor growth, high recurrence rates, and resistance against common chemotherapeutical regimes. The polyether-antibiotic Salinomycin is a promising drug in cancer therapy because of its ability to overcome apoptosis resistance of cancer cells and its selectivity against cancer stem cells. Here, we investigated the effectiveness of Salinomycin against cholangiocarcinoma in vivo, and analyzed interference of Salinomycin with autophagic flux in human cholangiocarcinoma cells...
January 9, 2018: Oncotarget
https://www.readbyqxmd.com/read/29421168/associations-of-urinary-phthalate-metabolites-and-lipid-peroxidation-with-sperm-mitochondrial-dna-copy-number-and-deletions
#15
Alexandra M Huffman, Haotian Wu, Allyson Rosati, Tayyab Rahil, Cynthia K Sites, Brian W Whitcomb, J Richard Pilsner
BACKGROUND: Phthalates, a chemical class of plasticizers, are ubiquitous environmental contaminants that have been associated with oxidative stress. Mitochondria DNA copy number (mtDNAcn) and DNA deletions (mtDNAdel) are emerging biomarkers for cellular oxidative stress and environment exposures. OBJECTIVES: To examine associations of urinary phthalate metabolite and isoprostane concentrations on sperm mtDNAcn and mtDNAdel in male partners undergoing assisted reproductive technologies (ART)...
February 5, 2018: Environmental Research
https://www.readbyqxmd.com/read/29420990/potential-roles-of-vitamin-e-in-age-related-changes-in-skeletal-muscle-health
#16
REVIEW
Eunhee Chung, Huanbiao Mo, Shu Wang, Yujiao Zu, Manal Elfakhani, Steven R Rios, Ming-Chien Chyu, Rong-Sen Yang, Chwan-Li Shen
Skeletal muscle disorders including sarcopenia are prevalent during the complex biological process of aging. Loss of muscle mass and strength commonly seen in sarcopenia is induced by impaired neuromuscular innervation, transition of skeletal muscle fiber type, and reduced muscle regenerative capacity, all attributable to chronic inflammation, oxidative stress, and mitochondrial dysfunction. Current literature suggests that vitamin E molecules (α-, β-, γ-, δ-tocopherols and the corresponding tocotrienols) with their antioxidant and anti-inflammatory capabilities may mitigate age-associated skeletal dysfunction and enhance muscle regeneration, thus attenuating sarcopenia...
January 2018: Nutrition Research
https://www.readbyqxmd.com/read/29417735/fetal-bovine-serum-can-reduce-toxicity-of-indocyanine-green-brilliant-blue-g-and-trypan-blue-in-arpe-19-cellular-model-that-suggests-new-surgical-staining-protocols-for-internal-limiting-membrane-peeling-procedure
#17
Yu-Yi Lin, Jorn-Hon Liu, Yin Chang
BACKGROUND: To investigate and compare the cytotoxicity of indocyanine green (ICG), brilliant blue G (BBG) and trypan blue (TB) using ARPE-19 cells that have been pre-treated/post- treated with balanced salt solution (BSS) or fetal bovine serum (FBS). METHODS: The cultured human retina pigment epithelium (RPE) ARPE-19 cells were pre-treated/post-treated with BSS or FBS (represent the autologous serum in clinic) in parallel with cells being soaked with various concentrations of ICG, BBG, and TB...
February 8, 2018: Clinical & Experimental Ophthalmology
https://www.readbyqxmd.com/read/29415895/intracellular-localization-of-diacylglycerols-and-sphingolipids-influences-insulin-sensitivity-and-mitochondrial-function-in-human-skeletal-muscle
#18
Leigh Perreault, Sean A Newsom, Allison Strauss, Anna Kerege, Darcy E Kahn, Kathleen A Harrison, Janet K Snell-Bergeon, Travis Nemkov, Angelo D'Alessandro, Matthew R Jackman, Paul S MacLean, Bryan C Bergman
BACKGROUND: Accumulation of diacylglycerol (DAG) and sphingolipids is thought to promote skeletal muscle insulin resistance by altering cellular signaling specific to their location. However,the subcellular localization of bioactive lipids in human skeletal muscle is largely unknown. METHODS: We evaluated subcellular localization of skeletal muscle DAGs and sphingolipids in lean individuals (n = 15), endurance-trained athletes (n = 16), and obese men and women with (n = 12) and without type 2 diabetes (n = 15)...
February 8, 2018: JCI Insight
https://www.readbyqxmd.com/read/29415881/tak1-regulates-skeletal-muscle-mass-and-mitochondrial-function
#19
Sajedah M Hindi, Shuichi Sato, Guangyan Xiong, Kyle R Bohnert, Andrew A Gibb, Yann S Gallot, Joseph D McMillan, Bradford G Hill, Shizuka Uchida, Ashok Kumar
Skeletal muscle mass is regulated by a complex array of signaling pathways. TGF-β-activated kinase 1 (TAK1) is an important signaling protein, which regulates context-dependent activation of multiple intracellular pathways. However, the role of TAK1 in the regulation of skeletal muscle mass remains unknown. Here, we report that inducible inactivation of TAK1 causes severe muscle wasting, leading to kyphosis, in both young and adult mice.. Inactivation of TAK1 inhibits protein synthesis and induces proteolysis, potentially through upregulating the activity of the ubiquitin-proteasome system and autophagy...
February 8, 2018: JCI Insight
https://www.readbyqxmd.com/read/29414393/complete-sequence-of-the-atp6-and-nd3-mitochondrial-genes-in-breast-cancer-tissue-of-postmenopausal-women-with-different-body-mass-indexes
#20
Mónica Martínez-Ramírez, Ramón Mauricio Coral-Vázquez, Alberto Tenorio, Juan Pablo Méndez, Jesús Benítez-Granados, Antonio Maffuz Asis, Sergio Rodríguez Cuevas, Carlos Domínguez Reyes, Aura Erazo-Valle, Patricia Canto
Due to the fact that mitochondrial defects and oxidative stress have been related with obesity and breast cancer is more aggressive in women with obesity, we investigated if postmenopausal Mexican-Mestizo women with breast cancer presented somatic mutations in the sequence of the ATP6 and/or ND3 genes. Twenty one postmenopausal Mexican-Mestizo women with breast cancer who underwent mastectomy or breast conserving surgery were studied. Height and weight were used to calculate body mass index. DNA from tumor tissue samples and blood leukocytes was amplified by polymerase chain reaction and sequenced the ATP6 and ND3 mitochondrial genes...
February 2018: Annals of Diagnostic Pathology
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