Search results for " Inclusion"

Familial mixed congenital myopathy with rigid spine phenotype

1997

We describe a father and daughter with a rigid spine syndrome and proximal myopathy. The index patient was a 42-year-old man, who died from respiratory failure after a lifelong, slowly progressive proximal myopathy and a rigid spine phenotype. This was morphologically characterized by cytoplasmic bodies, increased desmin, features of reducing-body myopathy, and sarcoplasmic and intranuclear tubulofilamentous inclusions. These cases are characterized by an early onset and possibly autosomal-dominant inheritance, with associated complex structural hallmarks of both desmin-related and inclusion body myopathies. Together they may be defined as a complex mixed congenital myopathy with a rigid sp…

The Tempered Polymerization of Human Neuroserpin

2012

Neuroserpin, a member of the serpin protein superfamily, is an inhibitor of proteolytic activity that is involved in pathologies such as ischemia, Alzheimer's disease, and Familial Encephalopathy with Neuroserpin Inclusion Bodies (FENIB). The latter belongs to a class of conformational diseases, known as serpinopathies, which are related to the aberrant polymerization of serpin mutants. Neuroserpin is known to polymerize, even in its wild type form, under thermal stress. Here, we study the mechanism of neuroserpin polymerization over a wide range of temperatures by different techniques. Our experiments show how the onset of polymerization is dependent on the formation of an intermediate mon…

Evidence of Two-Component Iblean-Like Mantle From 2001-2006 Igneous Products of Mount Etna

2008

EVIDENCES OF ENRICHED MANTLE (EM-2) SOURCE CONTRIBUTION TO ETNEAN MAGMAS: A COMPREHENSIVE STUDY ON FLUID AND MELT INCLUSIONS OF 2001-2006 ERUPTIONS

2009

Diversity in the City: New Planning Challenges in Inner Urban Areas of Southern Italy

2008

Progetto Tales at Home. APP-rendere attraverso il linguaggio delle emozioni nelle famiglie multilingue

2019

‘Tales at Home’ (Talking About Language and Emotions at Home) è un progetto Europeo finanziato nell’ambito della KA2 “Cooperation for innovation and the exchange of good praticies” del programma Erasmus+ 2015-2016 e nasce con l’idea di fornire strumenti e tecniche di supporto alle famiglie multilingue, al fine di favorire l’apprendimento delle lingue e la promozione del benessere in un’ottica di inclusione sociale. "Tales at Home" is a European project funded under KA2 “Cooperation for innovation and the exchange of good praticies "of the Erasmus + program 2015-2016 and is born with the idea to provide tools and support techniques to multilingual families, in order to foster language learni…

Conformational changes in Cmethyl-resorcinarene pyridine N -oxide inclusion complexes in the solid state

2016

Aromatic N-oxides interact with Cmethyl-resorcinarene resulting in marked changes in the conformation of the host resorcinarene. In the solid state, 2- and 3-methylpyridine N-oxides form pseudo-capsular 2 : 2 endo host-guest complexes with Cmethyl-resorcinarene stabilized by C-H⋯π interactions. The Cmethyl-resorcinarene·2-methylpyridine N-oxide complex has a C4v crown conformation, while the Cmethyl-resorcinarene·3-methylpyridine N-oxide complex has a slightly open C2v boat conformation. On the contrary, other para-substituted and benzo-fused pyridine N-oxides form only exo complexes with Cmethyl-resorcinarene. In the exo complexes, the asymmetry of the guest, conformational flexibility and…

Noble gases investigation in fluid inclusions of olivines and pyroxenes from Etnean products erupted in the 2001-2004 period.

2008

Dehydration of melt inclusions in olivine and implications for the origin of silica-undersaturated island-arc melts

2019

Highlights • New experiments with melt inclusions in olivine at 1200 °C and 300 MPa. • Coupled behavior H2O and SiO2 in inclusions during re-hydration and dehydration. • SiO2 mobility results from formation/destruction of metal vacant olivine. • SiO2-undersaturated arc melt inclusions may originate by dehydration. • New method to assess initial H2O in dehydrated inclusions. Abstract Primary subduction-related magmas build up modern continental crust and counterbalance massive recycling of crustal material into the deep mantle occurring at this tectonic setting. Melt inclusions in Mg-rich olivine are believed to be the best probes of primary subduction-related melts. However, unexpectedly, m…

Eclogite xenoliths from the Kuruman kimberlites, South Africa: geochemical fingerprinting of deep subduction and cumulate processes☆

2004

The mineralogically diverse eclogite xenolith suite sampled by the >1.6 Ga Zero kimberlite in the Kuruman group, close to the western edge of the Kaapvaal craton consists of bimineralic eclogites, orthopyroxene-bearing eclogites with and without sanidine, and kyanite eclogites. Garnet exsolutions in clinopyroxene occur in all Zero eclogite types, and orthopyroxene and, where present, sanidine are also exsolved from clinopyroxene in orthopyroxene-bearing eclogites. Mineral thermobarometry indicates that the last P–T condition prior to the kimberlite eruption was 900–950 °C at 3.5–4.0 GPa. However, inclusions of quartz, rutile and rarely K-feldspar, plus former fluid inclusions in garnets, in…