neuroprotection in amyotrophic lateral sclerosis

... tissue staining intensities influenced the measured values Average value density measurements from individual Vgf immunoreactive dorsal spinal cord neurons, reflecting immunostaining intensity, ... delimiting the cellular area of interest free hand, using predetermined criteria to define the region of interest The immune intensity of the cellular Vgf encompassing the L3-L5 regions of spinal ... C-terminal epitope of Vgf Unoccupied binding sites on the plates were blocked by incubation with casein Samples and standards were applied in duplicate and incubated overnight at 4°C Following...

Ngày tải lên: 03/11/2012, 10:52

... SDR family member Destrin (actin-depolymerizing factor) EF-hand domain containing protein 2, Swiprosin-1 Ezrin (p81) (cytovillin) (villin-2) Heat shock 27 kDa protein Integrin a-M precursor Lysosome-associated ... precursor Serine ⁄ threonine-protein phosphatase 2A catalytic subunit SH3 adapter protein SPIN90 (NCK-interacting protein with SH3 domain) Sushi domain-containing protein precursor FEBS Journal ... Fig Increased plasma membrane staining of annexin II in G93A motor neurons Immunofluorescent staining of annexin II and the neuronal marker neurofilament M (NF-M) in spinal cord motor neurons in...

Ngày tải lên: 23/03/2014, 04:21

Ngày tải lên: 19/06/2014, 22:20

... protein kinase N Polo-like kinase protein phosphatase protein phosphatase A phophatases Peptidyl-prolyl isomerases Proline phosphorylated serine/threonine-proline recombinant AAV RNA-interfering ... immunohistochemistry Interleukin induced nitric oxide synthase enzyme inositol 1,4,5-triphosphate receptor gene inverted terminal repeats JNK-interacting protein c-Jun N-terminal kinase Luria Bertani ... TAR DNA binding protein-43 tetramethylethylenediamine tangential flow filtration Transforming growth factor Threonine tumor necrosis factor  Tat-RNA-binding protein Telomeric Repeat-Binding Factor...

Ngày tải lên: 10/09/2015, 09:29

... binding sites in the spinal cord and GSH levels in cerebrospinal fluid were high in SALS patients [14,15], possibly because of a long-term response to chronic oxidative stress Mice overexpressing ... mutant protein, they maintained the redox equilibrium changing several enzymatic activities Wild-type and G93ASOD1 affect the levels of GCLC and GCLM proteins differently The increase in GSH in SOD1-transfected ... higher in highWT-tTA cells than in highG93A-tTA cells These mechanisms need to be investigated further The increase in GSH was long-lasting in lowG93AtTA cells, coupled with higher GCL activity In...

Ngày tải lên: 29/03/2014, 23:20

... chemokine ligand-2 (CCL2), a proinflammatory molecule, may impart neuroprotection in ALS against glutamate induced excitotoxicity either by reducing release of glutamate and/or increasing efficiency ... ALS patients could not be ascertained Since elevated CCL2 initiates inflammatory reaction by increasing production of nitric oxide and other inflammatory chemokines from unregulated monocytes/ ... growth factor-A and chemokine ligand (CCL2) genes are upregulated in peripheral blood mononuclear cells in Indian amyotrophic lateral sclerosis patients Journal of Neuroinflammation 2011 8:114...

Ngày tải lên: 19/06/2014, 22:20

... however is in agreement in both studies; the decrease of inosine in our study (Table 3) and the decline in the levels of metabolites of xanthine metabolism in the clinical study [80] point to a ... an effective therapy is a daunting uphill task with numerous agents failing in clinical trials despite showing efficacy in the preclinical disease endpoints Using Gas Chromatography/Time-Of-Flight ... atrophy resulting in paralysis ALS begins in the limbs, usually the arms, in about two-thirds of patients The first symptoms are most often unilateral and focal Early findings include foot drop,...

Ngày tải lên: 30/09/2015, 10:12

... clinical trial of verapamil in amyotrophic lateral sclerosis Muscle Nerve, Vol 19, No 4, pp 511-515 Miller, S.C & Warnick, J.E (1989) Protirelin (thyrotropin-releasing hormone) in amyotrophic lateral ... Pessina, G (1955) [Trypan red in therapy of amyotrophic lateral sclerosis and of myelopathic muscular atrophy] Rass Int Clin Ter, Vol 35, No 6, pp 197-206 Amyotrophic Lateral Sclerosis: An Introduction ... (2003) A double-blind randomized clinical trial in amyotrophic lateral sclerosis using lamotrigine: effects on CSF glutamate, aspartate, branched-chain amino acid levels and clinical parameters...

Ngày tải lên: 22/06/2014, 03:20

... the calcium-binding proteins calbindinD28K, calretinin, calmodulin and parvalbumin in the rat spinal cord Brain Res Brain Res Rev 19:163-179 Rintoul GL, Bennett VJ, Papaconstandinou NA, Reynolds ... (5HT) and norepinephrine (NE) have potent effects on motoneurons, including increasing PIC amplitude, decreasing input conductance, hyperpolarizing spike threshold, and depolarizing resting potential ... 182 Amyotrophic Lateral Sclerosis families from whom they were isolated (Deng et al., 2011) Even in sALS patients, ubiquilin2 was found in abnormal protein aggregates in degenerating neurons, indicating...

Ngày tải lên: 22/06/2014, 03:20

... determining step of folding involves overcoming the major energetic barrier to folding by forming a transition state complex prior to attaining the native state In more complex cases, protein folding ... Many factors are involved in modulating protein aggregation, and are surveyed in the following sections 1.2.1 Protein folding, stability and aggregation Globular protein folding begins on the ribosome, ... folding, before moving to the Zn-binding site (Nordlund et al 2009) Other studies have demonstrated that Zn-binding is required to 282 Amyotrophic Lateral Sclerosis pre-organize the Cu-binding...

Ngày tải lên: 22/06/2014, 03:20

... Gralla, and J S Valentine 1996 Mutations in copper-zinc superoxide 330 Amyotrophic Lateral Sclerosis dismutase that cause amyotrophic lateral sclerosis alter the zinc binding site and the redox ... site in human SOD1 that could enhance the coordination of the protein with immobilized Cu Indeed, C111S substitution eliminated the increase of Cu binding in mutant SOD1 Moreover, the protein degradation ... peroxynitrite-induced apoptosis of motor neurons and PC12 cells by tyrosinecontaining peptides J Biol Chem 282:6324-6337 14 Protein Aggregates in Pathological Inclusions of Amyotrophic Lateral Sclerosis...

Ngày tải lên: 22/06/2014, 03:20

... Enrichment of C-terminal fragments in TAR DNA-binding protein-43 cytoplasmic inclusions in brain but not in spinal cord of frontotemporal lobar degeneration and amyotrophic lateral sclerosis Am J ... key indicator is often the upregulation in IDO-1 resulting in an accelerated and sustained degradation in TRP The kynurenine pathway and QUIN in ALS The interest in the kynurenine pathway in the ... (1996b) Intense superoxide dismutase-1 immunoreactivity in intracytoplasmic hyaline inclusions of familial amyotrophic lateral sclerosis with Protein Aggregates in Pathological Inclusions of Amyotrophic...

Ngày tải lên: 22/06/2014, 03:20

... levels in fibroblasts isolated 468 Amyotrophic Lateral Sclerosis from an ALS patient A phase I clinical trial has been initiated in fALS patients with SOD1 mutations testing intrathecal infusion ... ameliorate amyotrophic lateral sclerosis in mice and correlate with disease progression in patients with amyotrophic lateral sclerosis Brain Vol 134, No Pt 5, pp 1293-314 Benatar, M., (2007) Lost in ... DNA/RNA binding protein that is involved in several cellular pathways including the splicing, transport and maturation of RNA (Lagier-Tourenne et al., 2010) FUS positive ubiquitinated cytoplasmic inclusions...

Ngày tải lên: 22/06/2014, 03:20

... nucleus and cytoplasm The 526 amino acid protein contains multiple protein domains, including a glutamineglycine-serine-tyrosine rich domain at the N-terminus, involved in transcriptional activation ... Novel optineurin mutations in sporadic amyotrophic lateral sclerosis patients Neurobiology of Aging, Ahead of print, ISSN 0197-4580 Vance C., et al (2006) Familial amyotrophic lateral sclerosis ... across the 15 exons encoding FUS; a third occur in exons 5-6 encoding the glycine-rich region and two thirds in exon 13-14 encoding the arginine-glycine-glycine (RGG)-rich domain and the nuclear localisation...

Ngày tải lên: 22/06/2014, 03:20

... (Print) 0261-5614 (Linking) 574 Amyotrophic Lateral Sclerosis Lou, J.S (2008) Fatigue in amyotrophic lateral sclerosis Phys Med Rehabil Clin N Am, Vol 19, No 3, pp 533-43, ix, 1047-9651 (Print) ... changes in amyotrophic lateral sclerosis Amyotroph Lateral Scler, Vol 8, No 1, pp 56-61, 1748-2968 (Print) 1471-180X (Linking) Hardiman, O (2007) Multidisciplinary care in motor neurone disease, In: ... 247-54, 03858146 (Print) 0385-8146 (Linking) Multidisciplinary Rehabilitation in Amyotrophic Lateral Sclerosis 573 Hillel, A.D & Miller R (1989) Bulbar amyotrophic lateral sclerosis: patterns...

Ngày tải lên: 22/06/2014, 03:20

... Methodology Chapter Amyotrophic Lateral Sclerosis: An Introduction to Treatment and Trials Martin H Maurer Chapter Insights Arising from Gene Expression Profiling in Amyotrophic Lateral Sclerosis Johnathan ... research, including data mining, gene expression profiling, and animal models In Chapter 1, Amyotrophic Lateral Sclerosis: An Introduction to Treatment and Trials” by Martin H Maurer, the author introduces ... reviewed In Chapter 15, “Involvement of the kynurenine pathway in amyotrophic lateral sclerosis , Guillemin Gilles introduces the kynurenine pathway as a major route for the catabolism of the amino...

Ngày tải lên: 28/06/2014, 11:20

... of amyotrophic lateral sclerosis Case Presentation: We present the case of a 57-year-old Caucasian man with pathological findings on postmortem examination consistent with amyotrophic lateral sclerosis ... signs should reinforce the possibility that amyotrophic lateral sclerosis may be present Since amyotrophic lateral sclerosis is a fatal illness, however, the possibility of this disease in patients ... by the Editor -in- Chief of this journal Competing interests The authors have no competing interests Authors’ contributions All of the authors were involved in drafting and revising the manuscript...

Ngày tải lên: 10/08/2014, 22:20

... an inability to extend the fingers Wrist flexion and finger flexion results were 4+/5 and interossei 3/5 In the right upper extremity the thenar group was 4-/5, infraspinatous 4/5, supraspinatous ... eventually resulting in death within years following parathyroidectomy There have been some interesting associations reported with ALS/MND, calcium and vitamin D metabolism In the 1970s Patten ... compared to the population in general [5] Despite an initial report suggesting that major improvements occurred sometimes coincidentally with reductions in paraprotein levels using prednisone, cyclophosphamide,...

Ngày tải lên: 11/08/2014, 02:21

... provided in this thesis would contribute to the understanding of the molecular mechanism underlying the mutation-causing disease 1.2 What is Amyotrophic lateral sclerosis? Amyotrophic lateral sclerosis ... Interactions with FFAT-motif containing proteins The hVAP proteins have been shown to interact with proteins carrying a short motif consisting of two phenylalanines in an acidic tract (Wyles et al., ... may be inert "packages" designed to protect the cell from toxic insult In this thesis, I focused on two point mutations in the MSP domain of VAPB that result in a form of protein folding disease...

Ngày tải lên: 12/10/2015, 17:34

... potentially increasing excitotoxic damage Calcineurin has been convincingly demonstrated to be involved in neuronal cell death since pharmacological experiments using the calcineurin inhibitors ... requires binding of a coagonist to the so-called glycine-binding site of the NMDA receptor Very recently, the amino acid D-serine has been suggested to be an endogenous ligand for the glycine-binding ... detected, indicating free radical production C, incubation with ascorbate resulted in detection of an ascorbyl radical signal following incubation in 14 mM Na+ and 2.5 µM Ca2+, supporting the finding...

Ngày tải lên: 08/04/2014, 12:51