Muscle pathology in myotonic dystrophy: light and electron microscopic investigation in eighteen patients

Myotonic dystrophy (DM) is the most common muscular dystrophy in adults. Two known genetic subtypes include DM1 (myotonic dystrophy type 1) and DM2 (myotonic dystrophy type 2). Genetic testing is considered as the only reliable diagnostic criterion in myotonic dystrophies. Relatively little is known about DM1 and DM2 myopathology. Thus, the aim of our study was to characterise light and electron microscopic features of DM1 and DM2 in patients with genetically proven types of the disease. We studied 3 DM1 cases and 15 DM2 cases from which muscle biopsies were taken for diagnostic purposes during the period from 1973 to 2006, before genetic testing became available at our hospital. The DM1 group included 3 males (age at biopsy 15–19). The DM2 group included 15 patients (5 men and 10 women, age at biopsy 26–60). The preferential type 1 fibre atrophy was seen in all three DM1 cases in light microscopy, and substantial central nucleation was present in two biopsies. Electron microscopy revealed central nuclei in all three examined muscle biopsies. No other structural or degenerative changes were detected, probably due to the young age of our patients. Central nucleation, prevalence of type 2 muscle fibres, and the presence of pyknotic nuclear clumps were observed in DM2 patients in light microscopy. Among the ultrastructural abnormalities observed in our DM2 group, the presence of internal nuclei, severely atrophied muscle fibres, and lipofuscin accumulation were consistent findings. In addition, a variety of ultrastructural abnormalities were identified by us in DM2. It appears that no single ultrastructural abnormality is characteristic for the DM2 muscle pathology. It seems, however, that certain constellations of morphological changes might be indicative of certain types of myotonic dystrophy. (Folia Morphol 2011; 70, 2: 121–129

A comparative analysis of cultural value orientations of Polish and Turkish employees:implications for international human resource management

This study empirically compares and contrasts the cultural value orientations of employees from Poland and Turkey by testing the compatibility of their values in three stages through seven cultural dimensions. The first phase of the study deals with the assessment of inter-country cultural value differences; the second phase investigates the intra-country cultural dynamics between selected demographic groups; and the third phase examines the inter-country cultural differences among the selected demographic groups of employees. The research has been conducted adopting the Maznevski, DiStephano, and Nason's (1995) version of cultural perspectives questionnaire with a sample of 744 (548 Polish and 196 Turkish) respondents. The results show significant cultural differences between Poland and Turkey, a presence of cultural dynamics among certain demographic groups within the country, and a mixture of convergence and divergence in the value systems of certain demographic groups both within and between the two nation(s). The research findings convey important messages to international human resource strategists in order for them to employ an effective and rational employment policy and business negotiation approach(es) to effectively operate in these countries. It also highlights that diversity of cultural values not only requires viewing each of them through cultural dimensions at a macro-level with a cross-country reference, but also requires monitoring their dynamics at the micro-level with reference to controlled demographic groups

N-Heterocyclic Carbene-Catalyzed Synthesis of Ynones via C–H Alkynylation of Aldehydes with Alkynyliodonium Salts

Alkynylation of aldehydes with alkynyl(aryl)iodonium salts catalyzed by an N-heterocyclic carbene (NHC) has been developed. The application of the organocatalyst and the hypervalent iodine group-transfer reagent allowed for metal-free C–H functionalization and C–C bond formation. The reaction proceeds under exceptionally mild conditions, at –40 ⁰C and in the presence of an amine base, providing access to an array of heteroaryl-propargyl ketones containing various substituents in good to excellent yields. The mechanism of the reaction was investigated by means of both experiments and density functional theory calculations. 13C-labelling and computations determined that the key alkynyl transfer step occurs via an unusual direct SN2 substitution of iodine-based leaving group by Breslow intermediate nucleophile at an acetylenic carbon. Moreover, kinetic studies revealed that the turnover-limiting step of the catalytic cycle is the generation of the Breslow intermediate, whereas the subsequent C–C bond-formation is a fast process. These results were fully reproduced and rationalized by the computed full free energy profile of the reaction, showing that the largest energy span is located between protonated NHC and the transition state for the carbene attack on the aldehyde substrate.&lt;br&gt;</jats:p

<i>N</i>‑Heterocyclic Carbene-Catalyzed Olefination of Aldehydes with Vinyliodonium Salts To Generate α,β-Unsaturated Ketones

An organocatalyzed metal-free, direct olefination of aldehydes with vinyliodonium salts has been achieved by an <i>N</i>-heterocyclic carbene-promoted C–H bond activation. The reaction proceeds under very mild conditions, delivering a range of (hetero)­aryl-vinyl ketones in good yields. The retention of the double bond configuration is uniformly observed, and the application of 2-methoxyphenyl auxiliary group in iodonium salts secures a complete selectivity of the vinyl transfer

N-Heterocyclic Carbene-Catalyzed Synthesis of Ynones via C–H Alkynylation of Aldehydes with Alkynyliodonium Salts

Alkynylation of aldehydes with alkynyl(aryl)iodonium salts catalyzed by an N-heterocyclic carbene (NHC) has been developed. The application of the organocatalyst and the hypervalent iodine group-transfer reagent allowed for metal-free C–H functionalization and C–C bond formation. The reaction proceeds under exceptionally mild conditions, at –40 ⁰C and in the presence of an amine base, providing access to an array of heteroaryl-propargyl ketones containing various substituents in good to excellent yields. The mechanism of the reaction was investigated by means of both experiments and density functional theory calculations. 13C-labelling and computations determined that the key alkynyl transfer step occurs via an unusual direct SN2 substitution of iodine-based leaving group by Breslow intermediate nucleophile at an acetylenic carbon. Moreover, kinetic studies revealed that the turnover-limiting step of the catalytic cycle is the generation of the Breslow intermediate, whereas the subsequent C–C bond-formation is a fast process. These results were fully reproduced and rationalized by the computed full free energy profile of the reaction, showing that the largest energy span is located between protonated NHC and the transition state for the carbene attack on the aldehyde substrate.<br /

Transition metal-free and regioselective vinylation of phosphine oxides and <em>H</em>-phosphinates with VBX reagents

A series of phosphine oxides and H-phosphinates were vinylated in the presence of the iodine(iii) reagents vinylbenziodoxolones (VBX), providing the corresponding alk-1-enyl phosphine oxides and alk-1-enyl phosphinates in good yields with complete chemo- and regioselectivity. The vinylation proceeds in open flask under mild and transition metal-free conditions.</p

Self-Supported Polymeric Ruthenium Complexes as Olefin Metathesis Catalysts in Synthesis of Heterocyclic Compounds

New ruthenium olefin metathesis catalysts containing N-heterocyclic carbene (NHC) connected by a linker tether to a benzylidene ligand were studied. Such obtained self-chelated Hoveyda&ndash;Grubbs type complexes existed in the form of an organometallic polymer but could still catalyze olefin metathesis after being dissolved in an organic solvent. Although these polymeric catalysts exhibited a slightly lower activity compared to structurally related nonpolymeric catalysts, they were successfully used in a number of ring-closing metathesis reactions leading to a variety of heterocyclic compounds, including biologically and pharmacologically related analogues of cathepsin K inhibitor and sildenafil (Viagra&trade;). In the last case, a good solubility of a polymeric catalyst in toluene allowed the separation of the product from the catalyst via simple filtration

N-Heterocyclic Carbene-Catalyzed Synthesis of Ynones via C–H Alkynylation of Aldehydes with Alkynyliodonium Salts—Evidence for Alkynyl Transfer via Direct Substitution at Acetylenic Carbon

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