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Frontotemporal lobar degeneration (FTLD) consists of a group of neurodegenerative diseases characterized by behavioural and executive impairment, language disorders and motor dysfunction. About 20-30 % of cases are inherited in a dominant manner. Mutations in the microtubule-associated protein tau gene (MAPT) cause frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17T). Here w
The catalytic cycle of multicopper oxidases (MCOs) involves intramolecular electron transfer (IET) from the Cu-T1 copper ion, which is the primary site of the one-electron oxidations of the substrate, to the trinuclear copper cluster (TNC), which is the site of the four-electron reduction of dioxygen to water. In this study we report a detailed characterization of the kinetic and electrochemical p
NADH:quinone oxidoreductase or complex I is a large membrane bound enzyme complex that has evolved from the combination of smaller functional building blocks. Intermediate size enzyme complexes exist in nature that comprise some, but not all of the protein subunits in full size 14-subunit complex I. The membrane spanning complex I subunits NuoL, NuoM and NuoN are homologous to each other and to tw
Background Non-heart-beating donor lung has been a promising source of lung transplantation. Many studies on non-heart-beating donor lungs are based on animal lung transplantation. In this study, we assessed by organ bath the effect of one-hour warm ischemia on the non-heart-beating donor lung in terms of the integrity of contractile and relaxant functions and tissue structures of pulmonic arterie
The shear-induced transformations between oriented planar lamellae and a state of closely packed multilamellar vesicles (MLVs) in a lyotropic nonionic surfactant model system were studied by the combination of nuclear magnetic resonance (NMR) spectroscopy and diffusometry with magnetic resonance imaging (MRI). H-2 NMR imaging confirmed the discontinuous nature of the transition from onions to plan
The random phase approximation (RPA) monopole strength and the unperturbed particle-hole excitation strength are studied, in which the strength in the continuum is properly treated without discretizing unbound particle spectra. The model is the self-consistent Hartree-Fock calculation and the RPA Green's function method with Skyrme interactions. Numerical examples are the Ni isotopes, especially N
A Monte-Carlo simulation package has been developed to model the response of a detector system for ion identification used in conjunction with ion separators following nuclear reactions. The simulation is written predominantly using the GEANT4 framework but utilises the ion transport code MOCADI for accurate separator and reaction modelling. A novel MOCADI-GEANT4 interface has been developed to ut
The pathological hallmark of Parkinson’s disease is dopaminergic neurodegeneration in the substantia nigra pars compacta neurons and accumulation of α-synuclein containing aggregates in the surviving neurons. The cause of cell death in Parkinson’s disease and the involvement of α-synuclein in the pathophysiology of the disease are unknown. Although other neuronal cell types exhibit α-synuclein pos
The K 2p photoionization and subsequent LMM Auger decay have been studied for molecular gas-phase KCl, KBr and KI. The detected manifold spectral structure of the Auger electron spectrum of KCl has been interpreted to originate from strong molecular field effects. This indicates that Auger decay on potassium side of originally very ionic molecules creates inner-shell hole states with a strong mole
Femtosecond laser pulse control of exciton dynamics in biological chromophore complexes is studied theoretically using the optimal control theory specified to open quantum systems. Based on the laser pulse induced formation of an excitonic wave packet the possibility to localize excitation energy at a certain chromophore within a photosynthetic antenna system (FMO complex of green bacteria) is inv
Background: The Btk (Bruton's tyrosine kinase) gene has been shown to be mutated in the human immunodeficiency disease, XLA (X-linked agammaglobulinemia). Btk is a member of the Tec family of cytosolic protein tyrosine kinases with distinct functional domains PH, TH, SH3, SH2, and kinase. Mutations have been observed in each of the Btk subdomains in XLA. We have analyzed the Btk gene in six XLA pa
We have examined long-term growth-factor expanded human neural progenitors following transplantation into the adult rat brain. Cells, obtained from the forebrain of a 9-week old fetus, propagated in the presence of epidermal growth factor, basic fibroblast growth factor, and leukemia inhibitory factor were transplanted into the striatum, subventricular zone (SVZ), and hippocampus. At 14 weeks, imp
Food-hoarding birds use memory to relocate caches, and species that store thousands of scattered food items must have an extraordinary memory capacity to be able to relocate them. Because the hippocampus is important in the functioning of spatial memory, it is logical to assume that the amount of food stored should correlate with hippocampal volume. Previously, food-hoarding capacity has been used
The potential use of in vitro-expanded precursor cells or cell lines in repair includes transplantation of such cells for cell replacement purposes and the activation of host cells to provide "self-repair." Recently, we have reported that cells from the brain-derived cell line RN33B (derived from the embryonic rat medullary raphe and immortalized through retroviral transduction of the temperature-
Osteochondritis dissecans is a disorder in which fragments of articular cartilage and subchondral bone dislodge from the joint surface. We analyzed a five-generation family in which affected members had autosomal-dominant familial osteochondritis dissecans. A genome-wide linkage analysis identified aggrecan (ACAN) as a prime candidate gene for the disorder. Sequence analysis of ACAN revealed heter
Background Several new therapeutic options for psoriasis have been tested in clinical trials in recent years. Choice of comparator, study duration and outcome measures are critical for interpreting application of trial results to clinical practice. Objectives We examined whether these trial aspects have changed substantially in recent years in comparison with the past. Methods A systematic search
The adsorption induced conformational changes of human carbonic anhydrase I (HCAi) and pseudo wild type human carbonic anhydrase II truncated at the 17th residue at the N-terminus (trHCAii) were studied in presence of nanoparticles of different sizes and polarities. Isothermal titration calorimetry (ITC) studies showed that the binding to apolar surfaces is affected by the nanoparticle size in com
The methanolic extract of Sesbania sesban Merrill (Fabaceae) raw seed materials contained total free phenolic content of 14.56 +/- 1.11 g catechin equivalent/100 g extract dry matter (DM). Encouraging levels of reducing power (FRAP, 1068 mmol Fe[II]/mg extract), inhibition of beta-carotene bleaching (49.80%) and scavenging activity against DPPH (51.65%) as well as superoxide (44.87%) radicals were
OBJECTIVES: Two functional genetic variants in the inosine triphosphatase (ITPA) gene have been shown to be strongly associated with protection from ribavirin (RBV)-induced hemolysis. We aimed at evaluating this finding in a chronic hepatitis C genotype 2/3 cohort with a predominance of genotype 3 patients where available data are scarce. A second objective was to determine whether a protective a
