نتایج جستجو برای: cuzn superoxide dismutase 1 sod1

تعداد نتایج: 2775979  

Journal: :Journal of neurology, neurosurgery, and psychiatry 2004
S Niemann H Joos T Meyer S Vielhaber U Reuner M Gleichmann R Dengler U Müller

Mutations in the gene encoding Cu/Zn superoxide dismutase (SOD1) account for approximately 20% of patients with familial amyotrophic lateral sclerosis (FALS). In this study, sequence analysis of exons 1-5 of SOD1 in a large German cohort with FALS was performed. Among 75 affected patients, who were not obviously related probands with a positive family history, nine had missense mutations in SOD...

Journal: :The Journal of biological chemistry 2006
Paul A Cobine Fabien Pierrel Megan L Bestwick Dennis R Winge

A mitochondrial matrix copper ligand (CuL) complex, conserved in mammalian cells, is the likely source of copper for assembly of cytochrome c oxidase (CcO) and superoxide dismutase 1 (Sod1) within the intermembrane space (IMS) in yeast. Targeting the copper-binding proteins human Sod1 and Crs5 to the mitochondrial matrix results in growth impairment on non-fermentable medium caused by decreased...

2014
Jae Keun Lee Sang Gil Hwang Jin Hee Shin Jaekyung Shim Eui-Ju Choi

Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disease with higher selectivity in degeneration of motor neurons. However, the molecular mechanism by which the ALS-linked mutants of human superoxide dismutase 1 (SOD1) gene induce neurotoxicity remains obscure yet. Here, we show that depletion of CIIA expression by RNA interference (RNAi) promoted cytotoxicity caused by A...

Journal: :Arteriosclerosis, thrombosis, and vascular biology 2004
Frank M Faraci Sean P Didion

Blood vessels express 3 isoforms of superoxide dismutase (SOD): cytosolic or copper-zinc SOD (CuZn-SOD), manganese SOD (Mn-SOD) localized in mitochondria, and an extracellular form of CuZn-SOD (EC-SOD). Because there are no selective pharmacological inhibitors of individual SOD isoforms, the functional importance of the different SODs has been difficult to define. Recent molecular approaches, p...

2015
Peter I. Joyce Philip Mcgoldrick Rachele A. Saccon William Weber Pietro Fratta Steven J. West Ning Zhu Sarah Carter Vinaya Phatak Michelle Stewart Michelle Simon Saumya Kumar Ines Heise Virginie Bros-Facer James Dick Silvia Corrochano Macdonnell J. Stanford Tu Vinh Luong Patrick M. Nolan Timothy Meyer Sebastian Brandner David L.H. Bennett P. Hande Ozdinler Linda Greensmith Elizabeth M.C. Fisher Abraham Acevedo-Arozena

Transgenic mouse models expressing mutant superoxide dismutase 1 (SOD1) have been critical in furthering our understanding of amyotrophic lateral sclerosis (ALS). However, such models generally overexpress the mutant protein, which may give rise to phenotypes not directly relevant to the disorder. Here, we have analysed a novel mouse model that has a point mutation in the endogenous mouse Sod1 ...

2004
Frank M. Faraci Sean P. Didion

Blood vessels express 3 isoforms of superoxide dismutase (SOD): cytosolic or copper-zinc SOD (CuZn-SOD), manganese SOD (Mn-SOD) localized in mitochondria, and an extracellular form of CuZn-SOD (EC-SOD). Because there are no selective pharmacological inhibitors of individual SOD isoforms, the functional importance of the different SODs has been difficult to define. Recent molecular approaches, p...

Journal: :Molecular pharmaceutics 2013
Jing Tong Xiang Yi Robert Luxenhofer William A Banks Rainer Jordan Matthew C Zimmerman Alexander V Kabanov

Superoxide dismutase 1 (SOD1) efficiently catalyzes dismutation of superoxide, but its poor delivery to the target sites in the body, such as brain, hinders its use as a therapeutic agent for superoxide-associated disorders. Here to enhance the delivery of SOD1 across the blood-brain barrier (BBB) and in neurons the enzyme was conjugated with poly(2-oxazoline) (POx) block copolymers, P(MeOx-b-B...

Adenilda Honorio-França, Alfredo Pereira-Junior, Bruno L Santos Carlos K B Ferrari Eduardo L França, Luciane A Monteiro

Objective(s): to investigate the chronopharmacological effects of growth hormone on executive function and the oxidative stress response in rats. Materials and Methods: Fifty male Wistar rats (36-40 weeks old) had ad libitum access to water and food and were separated into four groups: diurnal control, nocturnal control, diurnal GH-treated, and nocturnal GH-treated animals. Levels of Cu, Zn sup...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2008
Isaac M Chiu Adam Chen Yi Zheng Bela Kosaras Stefanos A Tsiftsoglou Timothy K Vartanian Robert H Brown Michael C Carroll

Amyotrophic Lateral Sclerosis (ALS) is an adult-onset, progressive, motor neuron degenerative disease, in which the role of inflammation is not well established. Innate and adaptive immunity were investigated in the CNS of the Superoxide Dismutase 1 (SOD1)(G93A) transgenic mouse model of ALS. CD4+ and CD8+ T cells infiltrated SOD1(G93A) spinal cords during disease progression. Cell-specific flo...

2016
Soumaya Ferchichi Hamdi Trabelsi Inès Azzouz Amel Hanini Ahmed Rejeb Olfa Tebourbi Mohsen Sakly Hafedh Abdelmelek

The purpose of our study was the evaluation of toxicological effects of silica-coated gold nanoparticles (GNPs) and static magnetic fields (SMFs; 128 mT) exposure in rat lungs. Animals received a single injection of GNPs (1,100 µg/kg, 100 nm, intraperitoneally) and were exposed to SMFs, over 14 days (1 h/day). Results showed that GNPs treatment induced a hyperplasia of bronchus-associated lymph...

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