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https://www.readbyqxmd.com/read/29777756/peroxiredoxin-5-regulates-adipogenesis-attenuating-oxidative-stress-in-obese-mouse-models-induced-by-a-high-fat-diet
#1
Mi Hye Kim, Sun-Ji Park, Jung-Hak Kim, Jung Bae Seong, Kyung-Min Kim, Hyun Ae Woo, Dong-Seok Lee
Elevated levels of reactive oxygen species (ROS) are a hallmark of obesity. Peroxiredoxin 5 (Prx5), which is a cysteine-dependent peroxidase enzyme, has an intensive ROS scavenging activity because it is located in the cytosol and mitochondria. Therefore, we focused on the role of Prx5 in regulating mitochondrial ROS and adipogenesis. We demonstrated that Prx5 expression was upregulated during adipogenesis and Prx5 overexpression suppressed adipogenesis by regulating cytosolic and mitochondrial ROS generation...
May 16, 2018: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/29777253/translation-inhibition-corrects-aberrant-localization-of-mutant-alanine-glyoxylate-aminotransferase-possible-therapeutic-approach-for-hyperoxaluria
#2
Ruth Belostotsky, Roman Lyakhovetsky, Michael Y Sherman, Fanny Shkedy, Shimrit Tzvi-Behr, Roi Bar, Bernd Hoppe, Björn Reusch, Bodo B Beck, Yaacov Frishberg
Primary hyperoxaluria type 1 is a severe kidney stone disease caused by abnormalities of the peroxisomal alanine-glyoxylate aminotransferase (AGT). The most frequent mutation G170R results in aberrant mitochondrial localization of the active enzyme. To evaluate the population of peroxisome-localized AGT, we developed a quantitative Glow-AGT assay based on the self-assembly split-GFP approach and used it to identify drugs that can correct mislocalization of the mutant protein. In line with previous reports, the Glow-AGT assay showed that mitochondrial transport inhibitors DECA and monensin increased peroxisomal localization of the mutant...
May 18, 2018: Journal of Molecular Medicine: Official Organ of the "Gesellschaft Deutscher Naturforscher und Ärzte"
https://www.readbyqxmd.com/read/29777059/sequence-diversity-of-tubulin-isotypes-in-regulation-of-the-mitochondrial-voltage-dependent-anion-channel
#3
Tatiana K Rostovtseva, Philip A Gurnev, David P Hoogerheide, Amandine Rovini, Minhajuddin Sirajuddin, Sergey M Bezrukov
The microtubule protein tubulin is a heterodimer comprising α/β subunits, in which each subunit features multiple isotypes in vertebrates. For example, seven α-tubulin and eight β-tubulin isotypes in the human tubulin gene family vary mostly in the length and primary sequence of the disordered anionic C-terminal tails (CTTs). The biological reason for such sequence diversity remains a topic of vigorous enquiry. Here, we demonstrate that it may be a key feature of tubulin's role in regulation of the permeability of the mitochondrial outer membrane voltage-dependent anion channel (VDAC)...
May 18, 2018: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29774306/leber-s-hereditary-optic-neuropathy-lhon-in-an-apulian-cohort-of-subjects
#4
Angelica Bianco, Luigi Bisceglia, Paolo Trerotoli, Luciana Russo, Leonardo D'Agruma, Silvana Guerriero, Vittoria Petruzzella
Leber's hereditary optic neuropathy (LHON) is a maternally inherited disorder that causes severe loss of sight in young adults, and is typically associated to mitochondrial DNA (mtDNA) mutations. Heteroplasmy of primary LHON mutations, presence of 'ancillary' mtDNA mutations, and mtDNA copy number are probably correlated with the penetrance and the severity of the disease. In this study, we performed a mutational screening in an Apulian cohort of LHON patients and we found that 41 out of 54 subjects harbored the m...
September 2017: Acta Myologica: Myopathies and Cardiomyopathies: Official Journal of the Mediterranean Society of Myology
https://www.readbyqxmd.com/read/29773917/neurodegenerative-disorders-rescuing-mitochondrial-motility
#5
Sarah Crunkhorn
No abstract text is available yet for this article.
May 18, 2018: Nature Reviews. Drug Discovery
https://www.readbyqxmd.com/read/29773347/lipophilic-methylene-blue-analogues-enhance-mitochondrial-function-and-increase-frataxin-levels-in-a-cellular-model-of-friedreich-s-ataxia
#6
Omar M Khdour, Indrajit Bandyopadhyay, Sandipan Roy Chowdhury, Nishant P Visavadiya, Sidney M Hecht
Friedreich's ataxia (FRDA) is an autosomal recessive neurodegenerative disorder resulting from reduced expression of the protein frataxin (FXN). Although its function is not fully understood, frataxin appears to help assemble iron sulfur clusters; these are critical for the function of many proteins, including those needed for mitochondrial energy production. Finding ways to increase FXN levels has been a major therapeutic strategy for this disease. Previously, we described a novel series of methylene violet analogues and their structural optimization as potential therapeutic agents for neurodegenerative and mitochondrial disorders...
May 4, 2018: Bioorganic & Medicinal Chemistry
https://www.readbyqxmd.com/read/29772520/-valeriana-wallichii-extract-inhibits-tert-booh-induced-oxidative-damage-and-cytotoxicity
#7
Vishal Jain, Ruma Dutta, Doddamane Thippeswamy Maheshwari, Darmendra Kumar Meena, Kshipra Misra, Mysore Sadashiva Yogendra Kumar
Oxidative s tress is well known to be involved in pathophysiology of several disorders. Valeriana wallichii (VW) root extracts is known for its expedient activities but its antioxidant and cytoprotective efficacy need to be explored further. In present study tertiary-butyl hydroperoxide (tert-BOOH) was used to induce oxidative stress in C6 glioma cells. Antioxidant activity of the VW root extracts were evaluated by chemical assays i.e . DPPH, ABTS and FRAP assay. Further effect of VW on tert-BOOH induced oxidative stress and mitochondrial damage was studied...
June 1, 2018: Frontiers in Bioscience (Elite Edition)
https://www.readbyqxmd.com/read/29771953/mitochondrial-disease-patient-motivations-and-barriers-to-participate-in-clinical-trials
#8
Zarazuela Zolkipli-Cunningham, Rui Xiao, Amy Stoddart, Elizabeth M McCormick, Amy Holberts, Natalie Burrill, Shana McCormack, Lauren Williams, Xiaoyan Wang, John L P Thompson, Marni J Falk
BACKGROUND: Clinical treatment trials are increasingly being designed in primary mitochondrial disease (PMD), a phenotypically and genetically heterogeneous collection of inherited multi- system energy deficiency disorders that lack effective therapy. We sought to identify motivating factors and barriers to clinical trial participation in PMD. METHODS: A survey study was conducted in two independent mitochondrial disease subject cohorts. A discovery cohort invited subjects with well-defined biochemical or molecularly- confirmed PMD followed at a single medical center (CHOP, n = 30/67 (45%) respondents)...
2018: PloS One
https://www.readbyqxmd.com/read/29767814/inborn-errors-of-coenzyme-a-metabolism-and-neurodegeneration
#9
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/29765450/lutein-prevents-osteoarthritis-through-nrf2-activation-and-downregulation-of-inflammation
#10
Yan-Qin Qiao, Pan-Feng Jiang, Yan-Zheng Gao
Introduction: Osteoarthritis is an inflammatory disorder associated with oxidative stress and apoptosis leading to cartilage destruction and impairment of cartilage formation. In the present study, we studied the protective effect of lutein against monosodium iodoacetate (MIA)-induced osteoarthritis in primary chondrocyte cells. Material and methods: Oxidative stress was determined through testing antioxidant status, reactive oxygen species levels and lipid peroxide content...
April 2018: Archives of Medical Science: AMS
https://www.readbyqxmd.com/read/29764717/mitochondrial-damage-and-cytoskeleton-reorganization-in-human-dermal-fibroblasts-exposed-to-artificial-visible-light-similar-to-screen-emitted-light
#11
Adeline Rascalou, Jérôme Lamartine, Pauline Poydenot, Frédéric Demarne, Nicolas Bechetoille
BACKGROUND: Artificial visible light is everywhere in modern life. Social communication confronts us with screens of all kinds, and their use is on the rise. We are therefore increasingly exposed to artificial visible light, the effects of which on skin are poorly known. OBJECTIVE: The purpose of this study was to model the artificial visible light emitted by electronic devices and assess its effect on normal human fibroblasts. METHODS: The spectral irradiance emitted by electronic devices was optically measured and equipment was developed to accurately reproduce such artificial visible light...
May 5, 2018: Journal of Dermatological Science
https://www.readbyqxmd.com/read/29763682/melatonin-attenuates-arsenic-induced-nephropathy-via-the-regulation-of-oxidative-stress-and-inflammatory-signaling-cascades-in-mice
#12
Sayanta Dutta, Sukanya Saha, Sushweta Mahalanobish, Pritam Sadhukhan, Parames C Sil
Arsenic is a potent inducer of several acute and chronic nephrotoxic disorders. It promotes deleterious phenomenon like oxidative stress, inflammation, cell death and altered glucose uptake leading to distorted kidney homeostasis that end up in chronic kidney disease. This study investigated the possible protective role of melatonin; a natural antioxidant produced by the pineal gland, against arsenic induced nephrotoxicity. Melatonin successfully ameliorated arsenic induced renal toxicity both in in vitro and in vivo models...
May 12, 2018: Food and Chemical Toxicology
https://www.readbyqxmd.com/read/29760952/downregulation-of-the-psychiatric-susceptibility-gene-cacna1c-promotes-mitochondrial-resilience-to-oxidative-stress-in-neuronal-cells
#13
Susanne Michels, Goutham K Ganjam, Helena Martins, Gerhard M Schratt, Markus Wöhr, Rainer K W Schwarting, Carsten Culmsee
Affective disorders such as major depression and bipolar disorder are among the most prevalent forms of mental illness and their etiologies involve complex interactions between genetic and environmental risk factors. Over the past ten years, several genome wide association studies (GWAS) have identified CACNA1C as one of the strongest genetic risk factors for the development of affective disorders. However, its role in disease pathogenesis is still largely unknown. Vulnerability to affective disorders also involves diverse environmental risk factors such as perinatal insults, childhood maltreatment, and other adverse pathophysiological or psychosocial life events...
2018: Cell Death Discovery
https://www.readbyqxmd.com/read/29759891/the-putative-role-of-oxidative-stress-and-inflammation-in-the-pathophysiology-of-sleep-dysfunction-across-neuropsychiatric-disorders-focus-on-chronic-fatigue-syndrome-bipolar-disorder-and-multiple-sclerosis
#14
REVIEW
Gerwyn Morris, Brendon Stubbs, Cristiano A Köhler, Ken Walder, Anastasiya Slyepchenko, Michael Berk, André F Carvalho
Sleep and circadian abnormalities are prevalent and burdensome manifestations of diverse neuro-immune diseases, and may aggravate the course of several neuropsychiatric disorders. The underlying pathophysiology of sleep abnormalities across neuropsychiatric disorders remains unclear, and may involve the inter-play of several clinical variables and mechanistic pathways. In this review, we propose a heuristic framework in which reciprocal interactions of immune, oxidative and nitrosative stress, and mitochondrial pathways may drive sleep abnormalities across potentially neuroprogressive disorders...
April 4, 2018: Sleep Medicine Reviews
https://www.readbyqxmd.com/read/29757967/epigenetics-of-subcellular-structure-functioning-in-the-origin-of-risk-or-resilience-to-comorbidity-of-neuropsychiatric-and-cardiometabolic-disorders
#15
REVIEW
Carlos Manuel Zapata-Martín Del Campo, Martín Martínez-Rosas, Verónica Guarner-Lans
Mechanisms controlling mitochondrial function, protein folding in the endoplasmic reticulum (ER) and nuclear processes such as telomere length and DNA repair may be subject to epigenetic cues that relate the genomic expression and environmental exposures in early stages of life. They may also be involved in the comorbid appearance of cardiometabolic (CMD) and neuropsychiatric disorders (NPD) during adulthood. Mitochondrial function and protein folding in the endoplasmic reticulum are associated with oxidative stress and elevated intracellular calcium levels and may also underlie the vulnerability for comorbid CMD and NPD...
May 14, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29753824/characterization-of-the-effects-of-trace-concentrations-of-graphene-oxide-on-zebrafish-larvae-through-proteomic-and-standard-methods
#16
Wei Zou, Qixing Zhou, Xingli Zhang, Li Mu, Xiangang Hu
The effects of graphene oxide (GO) carbon nanomaterials on ecosystems have been well characterized, but the toxicity of GO at predicted environmental concentrations to living organisms at the protein level remain largely unknown. In the present work, the adverse effects and mechanisms of GO at predicted environmental concentrations were evaluated by integrating proteomics and standard analyses for the first time. The abundances of 243 proteins, including proteins involved in endocytosis (e.g., cltcb, arf6, capzb and dnm1a), oxidative stress (e...
May 10, 2018: Ecotoxicology and Environmental Safety
https://www.readbyqxmd.com/read/29753771/mir-199a-3p-regulates-brown-adipocyte-differentiation-through-mtor-signaling-pathway
#17
Yao Gao, Yan Cao, Xianwei Cui, Xingyun Wang, Yahui Zhou, Fangyan Huang, Xing Wang, Juan Wen, Kaipeng Xie, Pengfei Xu, Xirong Guo, Lianghui You, Chenbo Ji
Recent discoveries of functional brown adipocytes in mammals illuminates their therapeutic potential for combating obesity and its associated diseases. However, on account of the limited amount and activity in adult humans of brown adipocyte depots, identification of miRNAs and characterization their regulatory roles in human brown adipogenesis are urgently needed. This study focused on the role of microRNA (miR)-199a-3p in human brown adipocyte differentiation and thermogenic capacity. A decreased expression pattern of miR-199a-3p was consistently observed during the differentiation course of brown adipocytes in mice and humans...
May 10, 2018: Molecular and Cellular Endocrinology
https://www.readbyqxmd.com/read/29753116/prolonged-treatment-with-mevalonolactone-induces-oxidative-stress-response-with-reactive-oxygen-species-production-mitochondrial-depolarization-and-inflammation-in-human-glioblastoma-u-87-mg-cells
#18
Rossella Gratton, Paola Maura Tricarico, Fulvio Celsi, Sergio Crovella
Mevalonate pathway impairment has been observed in diverse diseases, including Mevalonate Kinase Deficiency (MKD). MKD is a hereditary auto-inflammatory disorder, due to mutations at mevalonate kinase gene (MVK), encoding mevalonate kinase (MK) enzyme. To date, the most accredited MKD pathogenic hypothesis suggests that the typical MKD phenotypes might be due to a decreased isoprenoid production rather than to the excess and accumulation of mevalonic acid, as initially supported. Nevertheless, recent studies provide clear evidences that accumulating metabolites might be involved in MKD pathophysiology by exerting a toxic effect...
May 9, 2018: Neurochemistry International
https://www.readbyqxmd.com/read/29752989/a-novel-pathogenic-variant-in-the-marveld2-gene-causes-autosomal-recessive-non-syndromic-hearing-loss-in-an-iranian-family
#19
Afsaneh Taghipour-Sheshdeh, Fatemeh Nemati-Zargaran, Narges Zarepour, Parisa Tahmasebi, Nader Saki, Mohammad Amin Tabatabaiefar, Javad Mohammadi-Asl, Morteza Hashemzadeh-Chaleshtori
BACKGROUND AND AIMS: Hearing loss (HL) is the most common sensorineural disorder and one of the most common human defects. HL can be classified according to main criteria, including: the site (conductive, sensorineural and mixed), onset (pre-lingual and post-lingual), accompanying signs and symptoms (syndromic and non-syndromic), severity (mild, moderate, severe and profound) and mode of inheritance (Autosomal recessive, autosomal dominant, X-linked and mitochondrial). Autosomal recessive non-syndromic HL (ARNSHL) forms constitute a major share of the HL cases...
May 9, 2018: Genomics
https://www.readbyqxmd.com/read/29750912/ras-signalling-in-energy-metabolism-and-rare-human-diseases
#20
REVIEW
L Dard, N Bellance, D Lacombe, R Rossignol
The RAS pathway is a highly conserved cascade of protein-protein interactions and phosphorylation that is at the heart of signalling networks that govern proliferation, differentiation and cell survival. Recent findings indicate that the RAS pathway plays a role in the regulation of energy metabolism via the control of mitochondrial form and function but little is known on the participation of this effect in RAS-related rare human genetic diseases. Germline mutations that hyperactivate the RAS pathway have been discovered and linked to human developmental disorders that are known as RASopathies...
May 8, 2018: Biochimica et Biophysica Acta
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