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Mitochondrial disfunction

Olga Mazuryk, Oksana Krysiak-Foria, Agnieszka Żak, Franck Suzenet, Agata Ptak-Belowska, Tomasz Brzozowski, Grażyna Stochel, Małgorzata Brindell
The mechanism of cell death induced by the ruthenium polypyridyl complexes comprising two 4,7-diphenyl-1,10-phenanthroline ligands as well as one unmodified 2,2'-bipyridyl or modified with 2-nitroimidazole moiety attached by shorter (C3H6) or longer (C6H12) linker was investigated. Cytotoxicity and proliferation assays revealed that the studied Ru polypyridyl complexes are more toxic against human pancreas carcinoma PANC-1 cell line than normal human keratinocytes HaCaT with IC50 of 3-5μM. The Ru complexes despite accumulation in mitochondria do not lead to mitochondrial disfunction, though decreasing of mitochondrial Ca(2+) causes mitochondria membrane hyperpolarization...
April 1, 2017: European Journal of Pharmaceutical Sciences
Paolo Ascenzi, Alessandra di Masi, Loris Leboffe, Marco Fiocchetti, Maria Teresa Nuzzo, Maurizio Brunori, Maria Marino
In 2000, the third member of the globin family was discovered in human and mouse brain and named neuroglobin (Ngb). Ngb is a monomeric 3/3 globin structurally similar to myoglobin and to the α- and β-chains of hemoglobin, however it displays a bis-histidyl six-coordinate heme-Fe atom. Therefore, ligand binding to the Ngb metal center is limited from the dissociation of the distal His(E7)64-Fe bond. From its discovery, more than 500 papers on Ngb structure, expression, reactivity, and localization have been published to highlight its biochemical properties and its role(s) in health and disease...
December 2016: Molecular Aspects of Medicine
Jifeng Guo, Dan He, Lei Wang, Jifeng Kang, Nan Li, Xinxiang Yan, Beisha Tang
OBJECTIVE: To investigate the effect of the L10P mutation on the cellular mitochondrial disfunction. METHODS: Spectrophotometer, flow cytometry and electron microscope was utilized to examine cell viability, reactive oxygen species (ROS), mitochondrial transmembrane potential, complex I activity and mitochondrial morphous of the HEK293 monoclone cell lines, in which wild-type and L10P mutant DJ-1 protein are stably expressed. RESULTS: Compared with the cell lines expressing empty vector, we found the ROS levels were elevated, the cell viability, mitochondrial transmembrane potential, complex I activity were reduced in the cells expressing L10P mutant DJ-1 protein (P<0...
December 2015: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
Subhasish Mondal, Jay Jyoti Roy, Tanmoy Bera
Leishmaniasis is a growing health problem in many parts of the world partly due to drug resistance of the parasite. This study reports on the fisibility of studying mitochondrial properties of two forms of wild-type L. donovani through the use of selective inhibitors. Amastigote forms of L. donovani exhibited a wide range of sensitivities to these inhibitors. Mitochondrial complex II inhibitor thenoyltrifluoroacetone and FoF1-ATP synthase inhibitors oligomycin and dicyclohexylcarbodiimide were refractory to growth inhibition of amastigote forms, whereas they strongly inhibited the growth of promastigote forms...
October 2014: Journal of Bioenergetics and Biomembranes
Sema T Koz, Giyasettin Baydas, Suleyman Koz, Nevgul Demir, Viktor S Nedzvetsky
We aimed to study the effects of gingko biloba extract (EGb) on oxidative stress, astrocyte maturation and cognitive disfunction in offspring of hyperhomocysteinemic rats. Hyperhomocysteinemia was induced in the pregnant rats by administration of methionine (1 gr/kg body weight) dissolved in drinking water throughout pregnancy. One group of animals has received same amount of methionine plus 100 mg/kg/day EGb during pregnancy. On the postnatal day 1, half of the pups from all groups were sacrificed to study the lipid peroxidation (LPO) in different subfractions of brain...
July 2012: Phytotherapy Research: PTR
O A Buneeva, A E Medvedev
Mitochondrial structural and functional abnormalities in Parkinson's disease and experimental animal models of this pathology are described. Special attention is paid to the inactivation of mitochondrial enzymes, mutations in mitochondrial and nuclear DNA, and genomic and proteomic research of mitochondrial proteins in Parkinson's disease and experimental parkinsonism of animals.
May 2011: Biomedit︠s︡inskai︠a︡ Khimii︠a︡
Antonio Pizzuti, Simona Petrucci
Recent studies on patient with sporadic ALS and on in vitro and in vivo models of mendelian diseases have been addressed toward the unravelling of the mitochondrial behaviour in ALS, whether as a primarily pathogenic factor, or as a fundamental contributor to the cell death. Morphological evidence suggests mitochondria pathology in ALS and many physiological mechanisms involving these organelles appear deranged in ALS, such as energy production, apoptotic triggering, calcium homeostasis and axonal transport of mitochondria...
March 2011: Archives Italiennes de Biologie
Sumana Mallick, Papiya Ghosh, Suman K Samanta, Sumita Kinra, Bikas C Pal, Aparna Gomes, Joseph R Vedasiromoni
PURPOSE: The presence of triterpene saponins in Corchorus acutangulus Lam. has been reported. However, no studies concerning biological activity of the plant extracts have been done so far. In the present study, the anti-leukemic activity of the methanol extract of aerial parts (ME) of C. acutangulus has been investigated, and efforts have been made to identify the active ingredient responsible for this activity. METHODS: The anti-leukemic activity of ME, its fractions and corchorusin-D (COR-D), the active ingredient, was investigated in leukemic cell lines U937 and HL-60 using cell viability and MTT assays...
September 2010: Cancer Chemotherapy and Pharmacology
Eduardo H B Delgado, Emilio L Streck, João L Quevedo, Felipe Dal-Pizzol
Malathion is a pesticide used on a large scale and with high potential risk for human exposure. However, it is reasonable to hypothesize that while the malathion is metabolizing reactive oxygen species (ROS) can be generated and subsequently there is onset of an oxidative stress in central nervous system (CNS) structures: hippocampus, cortex, striatum and cerebellum of intoxicated rats due to mitochondrial respiratory chain disfunctions. The present study was therefore undertaken to evaluate malathion-induced lipid peroxidation (LPO), superoxide production from sub-mitochondrial particles and the activity of complexes II and IV of the mitochondrial respiratory chain...
August 2006: Neurochemical Research
G Malet, A G Martín, M Orzáez, M J Vicent, I Masip, G Sanclimens, A Ferrer-Montiel, I Mingarro, A Messeguer, H O Fearnhead, E Pérez-Payá
Apoptosis is a biological process relevant to human disease states that is strongly regulated through protein-protein complex formation. These complexes represent interesting points of chemical intervention for the development of molecules that could modulate cellular apoptosis. The apoptosome is a holoenzyme multiprotein complex formed by cytochrome c-activated Apaf-1 (apoptotic protease-activating factor), dATP and procaspase-9 that link mitochondria disfunction with activation of the effector caspases and in turn is of interest for the development of apoptotic modulators...
September 2006: Cell Death and Differentiation
Li-li Cao, Guan-hua Du, Min-wei Wang
AIM: To study the protective effect of salidroside on mitochondria injury induced by sodium azide. METHODS: Human neuroblastoma SH-SY5Y cells were exposed to sodium azide with different concentration of salidroside, then cell viability was measured by thiazolyl blue (MTT) method and mitochondrial membrane potential (MMP) was detected by JC-1 method. Protective effect of salidroside against disfunction of mitochondria induced by sodium azide was detected by resazurin method...
August 2005: Yao Xue Xue Bao, Acta Pharmaceutica Sinica
A V Dmytriieva, V F Sahach, A Iu Bohuslavs'kyĭ
In experiments on the isolated myocardial and vascular preparations the role of the mithochondrial permiability transition pore (mPTP) in the development of reperfusion injury was investigated. Co-perfusion of the previously activated myocardial trabecula (MT) and arterial rings (AR) by solution collected during the first 5 min of isolated heart reperfusion, caused a sharp and significant decrease of tonic tension of both isolated preparations. Besides the significant inhibition of the MT and AR reactions after electrical stimulation, modulation of AR reaction by the influence of MT is also registered...
2005: Fiziolohichnyĭ Zhurnal
M Candelli, I A Cazzato, M A Zocco, E C Nista, L Fini, A Armuzzi, V Camise, M Santoro, L Miele, A Grieco, G Gasbarrini, A Gasbarrini
Breath tests for "dynamic" liver function evaluation have been proposed several years ago. A variety of carbon-labelled breath tests for the assessment of mitochondrial, microsomal and cytosolic liver function have been described with the aim to increase data on liver disease staging, prognosis, and response to therapy. In the last years a great interest is developed about the use of breath test for liver mitochondrial function evaluation since it results impaired in a wide range of liver diseases either of genetic or acquired origin...
January 2004: European Review for Medical and Pharmacological Sciences
Yasukazu Saitoh, Rika Ouchida, Atsushi Kayasuga, Nobuhiko Miwa
Although it is well known that Bcl-2 can prevent apoptosis, the Bcl-2's anti-apoptotic mechanism is not fully understood. Here, we investigate the mechanism of oxidant-induced cell death and to investigate the role of Bcl-2 in the tert-butyl hydroperoxide (t-BuOOH)-induced oxidant injury in Rat-1 fibroblasts and their bcl-2 transfected counterparts, b5 cells. Treatment with t-BuOOH causes mitochondrial disfunction and induced morphological features consistent with apoptosis more markedly in Rat-1 cells than in b5 cells...
May 15, 2003: Journal of Cellular Biochemistry
Motoya Sato, Hiroshi Ohguro
No abstract text is available yet for this article.
April 2002: Nihon Rinsho. Japanese Journal of Clinical Medicine
J Fernandes Leite
INTRODUCTION: Huntington s disease is a genetic autosomal dominant progressive neurodegenerative disorder determined by mutation at the gene that codes for the protein huntingtin, whose function is unknown. Clinically hallmarked by chorea and behavioral disturbances, the diagnosis is confirmed by blood test for the disease s gene. Experimental models of the disease, and new tools for in vivo and in vitro investigation are contributing for understanding its pathophysiology. DEVELOPMENT: The neurodegeneration is accomplished by apoptosis and predominantly strikes the striatum...
April 16, 2001: Revista de Neurologia
M R Vilà, J Gámez, A Solano, A Playán, S Schwartz, F M Santorelli, C Cervera, C Casali, J Montoya, F Villarroya
Multiple symmetric lipomatosis (MSL) is a rare disorder characterised by large subcutaneous fat masses in some parts of the trunk. Mitochondrial disfunction is common in MSL, but the identity of the adipose cells developing in multiple lipomas is not well known. We determined that brown adipose tissue-specific uncoupling protein-1 (UCP-1) mRNA is expressed in the lipoma of a multiple symmetric lipomatosis patient bearing the 8344 mutation in the tRNALys gene of mitochondrial DNA. UCP1 mRNA was not detected in normal subcutaneous fat from the same patient or in the lipoma of another patient bearing a different mutation in the same tRNALys gene...
November 30, 2000: Biochemical and Biophysical Research Communications
S Blanche, M Tardieu, P Rustin
No abstract text is available yet for this article.
January 2000: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
E Franco, J Bautista, R Luque, I Chinchón, R García-Lozano, I Aguilera, Y Campos, J Arenas
A 60-year-old woman had developed ptosis, progressive external ophthalmoplegia and action tremor over the last ten years. Physical examination also revealed short stature and retinal pigmentation. Anaerobic forearm exercise test showed increase of basal lactate and rise of lactate/piruvate index. Biceps biopsy displayed numerous ragged red fibers. Respiratory chain studies were consistent with complex I deficiency. Point mutations or deletions in mitochondrial DNA were not found. MR identified a diffuse leukoencephalopathy over both cerebral hemispheres, mesencephalon, pons and cerebellum...
November 1999: Neurología: Publicación Oficial de la Sociedad Española de Neurología
V D Longo, L L Liou, J S Valentine, E B Gralla
Yeast lacking mitochondrial superoxide dismutase (MnSOD) display shortened stationary-phase survival and provide a good model system for studying mitochondrial oxidative damage. We observed a marked decrease in respiratory function preceding stationary-phase death of yeast lacking MnSOD (sod2Delta). Agents (mitochondrial inhibitors) that are known to increase or decrease superoxide production in submitochondrial particles affected stationary-phase survival in a manner inversely correlated with their effects on superoxide production, implicating superoxide in this mitochondrial disfunction...
May 1, 1999: Archives of Biochemistry and Biophysics
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