Search results for "Wester"

Translation of HTT mRNA with expanded CAG repeats is regulated by the MID1-PP2A protein complex.

2012

Expansion of CAG repeats is a common feature of various neurodegenerative disorders, including Huntington's disease. Here we show that expanded CAG repeats bind to a translation regulatory protein complex containing MID1, protein phosphatase 2A and 40S ribosomal S6 kinase. Binding of the MID1-protein phosphatase 2A protein complex increases with CAG repeat size and stimulates translation of the CAG repeat expansion containing messenger RNA in a MID1-, protein phosphatase 2A- and mammalian target of rapamycin-dependent manner. Our data indicate that pathological CAG repeat expansions upregulate protein translation leading to an overproduction of aberrant protein and suggest that the MID1-com…

Sentimentalismi ja virheteoria Edvard Westermarckin moraalifilosofiassa

2015

Tutkimuksen tarkoituksena on selvittää voidaanko Edvard Westermarckin moraalifilosofiaa käsitellä nykyisessä metaetiikassa virheteorian ja sentimentalismin näkökulmasta. Tärkeäksi valitun näkökulman tekee se, että erityisesti Westermarckin esittämään virheteoriaan on kiinnitetty vähän huomiota. Sentimentalismin ja virheteorian käsittelyn lisäksi selvennän Westermarckin käsitystä subjektivismista ja relativismista, sillä Westermarckin on katsottu edustavan yksinkertaista subjektivismia tai kestämätöntä relativismia. Väitän, että nämä käsitykset ovat hänen moraalifilosofiasta riittämättömiä. Argumentoin Westermarckiin kohdistuneen kritiikin perustuvan pääosin väärinymmärrykselle tai virheelli…

Ideologiczne i polityczne aspekty klasycznego westernu na przykładzie "Winchester`73" oraz "Gwiazda szeryfa" Antony`ego Manna

2016

The article presents political analysis of two classic westerns directed by Anthony Mann - "Winchester 73" and "Tin Star". The author took up three problems. Firstly, westerns are treated as political myths functioning in the collective consciousness by which communities can handle universal political problems (e.g. relations between individuaI and society; fundamental conditions of public order). Secondly, the contextual analysis of both films has been conducted. Despite many similarities between the movies (the same genre, the same creative concept, the same narrative scheme), both of them differ ideologically. "Winchester'73" (1950)expresses the conservative message while "Tin Star" the …

Tiedolla vaikuttaja

2018

Evaluation of waterborne exposure to heavy metals in innate immune defences present on skin mucus of gilthead seabream (Sparus aurata)

2015

Aquatic animals are continuously exposed to chemical pollutants but the effects evoked in skin surfaces, which receive the most direct contact with them, are poorly investigated. Terminal carbohydrate composition and immunological components present in skin mucus of gilthead seabream (Sparus aurata L.) specimens exposed to waterborne sublethal dosages of heavy metals [arsenic (As2O3), cadmium (CdCl2) and mercury (CH3HgCl) at 5, 5 and 0.04 μM, respectively for 2, 10 and 30 days were analysed. Moreover, the presence of a fucose binding lectin (FBL) was evaluated by western blot and the protein profiles were by SDS-PAGE and HPLC. Results showed little effects of heavy metals in the presence of…

Abnormalities in alpha-dystroglycan expression in MDC1C and LGMD2I muscular dystrophies

2004

We recently identified mutations in the fukutin related protein (FKRP) gene in patients with congenital muscular dystrophy type 1C (MDC1C) and limb girdle muscular dystrophy type 2I (LGMD2I). The sarcolemma of these patients typically displays an immunocytochemical reduction of alpha-dystroglycan. In this report we extend these observations and report a clear correlation between the residual expression of alpha-dystroglycan and the phenotype. Three broad categories were identified. Patients at the severe end of the clinical spectrum (MDC1C) were compound heterozygote between a null allele and a missense mutation or carried two missense mutations and displayed a profound depletion of alpha-d…

Expression of 60-kD Heat Shock Protein Increases during Carcinogenesis in the Uterine Exocervix

2002

<i>Objectives:</i> The aim of the present study was to determine the presence and expression of the 60-kD heat shock protein (HSP60) in the dysplasia-carcinoma sequence in the uterine exocervix and to evaluate its diagnostic and prognostic significance. <i>Methods and Results:</i> We performed Western blot and immunohistochemical analyses on biopsies from 40 cases, consisting of 10 normal exocervical biopsies, 10 low-grade squamous intraepithelial lesions (L-SIL), 10 high-grade squamous intraepithelial lesions (H-SIL) and 10 cancerous exocervices (G2 grade). The immunohistochemical results were quantified by computer-assisted image analysis. Western blot analysis sho…

The unspoken pressure of tradition : East Asian classical musicians in western classical music

2013

Itäaasialaisia klassisia muusikoita käsittelevät artikkelit ovat olleet yleisiä eurooppalaisissa klassisen musiikin lehdissä siitä lähtien kun itäaasialaiset alkoivat yhä suuremmassa määrin voittaa kansainvälisiä musiikkikilpailuita ja luoda kansainvälistä uraa 1900-luvun viimeisinä vuosikymmeninä. Läntinen taidemusiikki on kansainvälistymässä aikana, jolloin pelot klassisen musiikin kuolemasta ovat myös päässeet otsikoihin Euroopassa. Itäaasialaiset muusikot työskentelevät kompleksisella kulttuurien kentällä, jossa heidän muusikkoutensa on jatkuvasti arvostelun alaisena. Tämän tutkimuksen tarkoitus oli saada selville, miten itäaasialaiset klassiset muusikot ja heidän kulttuurinen identitee…

Myopathy with hexagonally cross-linked crystalloid inclusions: delineation of a clinico-pathological entity.

2010

A novel myopathy characterized by hexagonally cross-linked tubular arrays has been reported in five patients. We studied the clinical and histopathological features of five additional unrelated patients with this myopathy. Patients experienced exercise intolerance with exercise-induced myalgia and weakness, without rhabdomyolysis. One patient additionally presented mild permanent pelvic girdle muscle weakness. Age at onset varied between 13 and 56 years. The inclusions were eosinophilic on H and E, bright red with modified Gomori’s trichrome stains, present in type 2 fibers, and revealed immunoreactivity selectively for a caveolin-3-antibody. Ultrastructurally, the inclusions showed a highl…

Eight new mutations and the expanding phenotype variability in muscular dystrophy caused by ANO5.

2012

Objective: Description of 8 new ANO5 mutations and significant expansion of the clinical phenotype spectrum associated with previously known and unknown mutations to improve diagnostic accuracy. Methods: DNA samples of 101 patients in 95 kindreds at our quaternary referral center in Finland, who had undetermined limb-girdle muscular dystrophy (LGMD), calf distal myopathy, or creatine kinase (CK) elevations of more than 2,000 IU/L, were selected for ANO5 genetic evaluation, and the clinical findings of patients with mutations were retrospectively analyzed. Results: A total of 25 patients with muscular dystrophy caused by 11 different recessive mutations in the ANO5 gene were identified. The …