Biochemical and Neuropathological Findings in a Creutzfeldt–Jakob Disease Patient with the Rare Val180Ile-129Val Haplotype in the Prion Protein Gene

Genetic Creutzfeldt–Jakob disease (gCJD) associated with the V180I mutation in the prion protein (PrP) gene (PRNP) in phase with residue 129M is the most frequent cause of gCJD in East Asia, whereas it is quite uncommon in Caucasians. We report on a gCJD patient with the rare V180I-129V haplotype, showing an unusually long duration of the disease and a characteristic pathological PrP (PrP^Sc) glycotype. Family members carrying the mutation were fully asymptomatic, as commonly observed with this mutation. Neuropathological examination showed a lesion pattern corresponding to that commonly reported in Japanese V180I cases with vacuolization and gliosis of the cerebral cortexes, olfactory areas, hippocampus and amygdala. PrP was deposited with a punctate, synaptic-like pattern in the cerebral cortex, amygdala and olfactory tract. Western blot analyses of proteinase-K-resistant PrP showed the characteristic two-banding pattern of V180I gCJD, composed of mono- and un-glycosylated isoforms. In line with reports on other V180I cases in the literature, Real-Time Quaking Induced Conversion (RT-QuIC) analyses did not demonstrate the presence of seeding activity in the cerebrospinal fluid and olfactory mucosa, suggesting that this haplotype also may result in a reduced seeding efficiency of the pathological PrP. Further studies are required to understand the origin, penetrance, disease phenotype and transmissibility of 180I-129V haplotype in Caucasians.     

Robust Identification: An approach to select the class of candidate models

A practical approach to assess the trade-offs in selecting the parameters that define the class of candidate models and that are commonly used in the Robust Identification framework is derived. The procedure minimizes the worst case identification error bound and guarantees consistency, according to all the experimental evidence. A consistency curve is defined, and upper and lower bounds are computed to graphically select these parameters.     

Hepatitis C virus and immunoglobulin gene rearrangements: an early step in lymphomagenesis?

The present status of Bcl-2 family proteins action and their role in leukemia and lymphoma is reviewed here in short. The Bcl-2 is an oncogenic protein that acts by inhibiting programmed cell death (apoptosis). In this article a timely review of the emerging mechanisms by which Bcl-2 and homologous family proteins might suppress cell death is presented. There have been reports that Bcl-2 and related anti-apoptotic proteins can function as a channel in the mitochondrial membrane and as an adaptor protein that can protect cells from cytotoxic agents. A dual function now seems likely, and interactions between Bcl-2 and other proteins are supposed. The Bcl-2 family proteins have assumed an important role in leukemia and lymphoma research. The observations reviewed in this article suggest an important role of dysregulated Bcl-2 expression in the pathogenesis and prognosis of at least some types of leukemia and lymphoma. The Bcl-2 family proteins are important regulators of apoptosis that constitute a novel mechanism of chemoresistance in cancer.     

A Supramolecular Strategy to Leverage the Liquid‐Phase Exfoliation of Graphene in the Presence of Surfactants: Unraveling the Role of the Length of Fatty Acids

Achieving the full control over the production as well as processability of high‐quality graphene represents a major challenge with potential interest in the field of fabrication of multifunctional devices. The outstanding effort dedicated to tackle this challenge in the last decade revealed that certain organic molecules are capable of leveraging the exfoliation of graphite with different efficiencies. Here, a fundamental understanding on a straightforward supramolecular approach for producing homogenous dispersions of unfunctionalized and non‐oxidized graphene nanosheets in four different solvents is attained, namely N‐methyl‐2‐pyrrolidinone, N,N‐dimethylformamide, ortho‐dichlorobenzene, and 1,2,4‐trichlorobenzene. In particular, a comparative study on the liquid‐phase exfoliation of graphene in the presence of linear alkanes of different lengths terminated by a carboxylic‐acid head group is performed. These molecules act as graphene dispersion‐stabilizing agents during the exfoliation process. The efficiency of the exfoliation in terms of concentration of exfoliated graphene is found to be proportional to the length of the employed fatty acid. Importantly, a high percentage of single‐layer graphene flakes is revealed by high‐resolution transmission electron microscopy and Raman spectroscopy analyses. A simple yet effective thermodynamic model is developed to interpret the chain‐length dependence of the exfoliation yield. This approach relying on the synergistic effect of a ad‐hoc solvent and molecules to promote the exfoliation of graphene in liquid media represents a promising and modular strategy towards the rational design of improved dispersion‐stabilizing agents.     

Locomotor function in the early stage of Parkinson's disease

The cardinal motor symptoms of Parkinson's disease (PD) have been widely investigated with particular reference to abnormalities of steady-state walking. The great majority of studies, however are related to severe forms of PD patients (phases > = 3 of Hoehn and Yahr scale), where locomotor abnormalities are clearly manifested. Goal of the present study was to quantitatively describe locomotor symptoms in subjects with mild PD. Accordingly, a multitask protocol involving instrumental analysis of steady-state linear walking, initiation of gait, and turning while walking was applied to a group of patients with idiopathic PD in their early clinical stage (phases 1 and 2 of Hoehn and Yahr scale), as well as in age-matched elderly controls. Kinematic, kinetic, and myoelectric measures were obtained by optoelectronic motion analysis, force platform, and telemetric electromyography. Results in PD patients showed a tendency to bradykinetic gait, with reduction of walking speed and cadence. Impairments of gait initiation consisted in reduction of the backward shift of the center of pressure (CoP) and prolongation of the stepping phase. Alterations of the turning task were more consistent and included delayed reorientation of the head toward the new direction, altered head-upper trunk rotational strategy, and adoption of a greater number of steps to complete the turning. It is concluded that patients in the early stage of PD reveal mild alterations of steady-state linear walking and more significant anomalies in the transitional conditions, especially during changes in the travel direction. Quantitative analysis of nonstationary locomotor tasks might be a potentially useful starting point for further studies on the pathophysiology of PD.     

Preparation of biodegradable polyesters/high‐amylose‐starch composites by reactive blending and their characterization

Two different biodegradable polyesters [polycaprolactone (PCL) and poly(3‐hydroxybutyrate‐co‐valerate) (PHBV)] were blended with a maize starch that had high amylose content through the use different reactive approaches. The compatibilization of both systems was obtained. PCL/starch composites were obtained by the addition of a third reactive component that was able to act as a coupling agent, and the reactive interface of PHBV/starch composites was improved during blending with an organic peroxide. Thermal, morphological, and mechanical characterization showed that the compatibilized composite materials had better final proprieties than neat materials or composites prepared without compatibilization. Finally, the degradation of all prepared materials by a compost simulation test was investigated. © 2002 Wiley Periodicals, Inc. J Appl Polym Sci 83: 1432–1442, 2002     

Recent trends in detecting,controlling, and detoxifying of patulin mycotoxin using biotechnology methods

Patulin (PAT) is a mycotoxin that can contaminate many foods and especially fruits and fruit‐based products. Therefore, accurate and effective testing is necessary to enable producers to comply with regulations and promote food safety. Traditional approaches involving the use of chemical compounds or physical treatments in food have provided practical methods that have been used to date. However, growing concerns about environmental and health problems associated with these approaches call for new alternatives. In contrast, recent advances in biotechnology have revolutionized the understanding of living organisms and brought more effective biological tools. This review, therefore, focuses on the study of biotechnology approaches for the detection, control, and mitigation of PAT in food. Future aspects of biotechnology development to overcome the food safety problem posed by PAT were also examined. We find that biotechnology advances offer novel, more effective, and environmental friendly approaches for the control and elimination of PAT in food compared to traditional methods. Biosensors represent the future of PAT detection and use biological tools such as aptamer, enzyme, and antibody. PAT prevention strategies include microbial biocontrol, the use of antifungal biomolecules, and the use of microorganisms in combination with antifungal molecules. PAT detoxification aims at the breakdown and removal of PAT in food by using enzymes, microorganisms, and various adsorbent biopolymers. Finally, biotechnology advances will be dependent on the understanding of fundamental biology of living organisms regarding PAT synthesis and resistance mechanisms.