202 research outputs found

    Medieval reformations

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    Book synopsis: n the last two decades, the history of the Counter-Reformation has been stretched and re-shaped in numerous directions. Reflecting the variety and innovation that characterize studies of early modern Catholicism today, this volume incorporates topics as diverse as life cycle and community, science and the senses, the performing and visual arts, material objects and print culture, war and the state, sacred landscapes and urban structures. Moreover, it challenges the conventional chronological parameters of the Counter-Reformation and introduces the reader to the latest research on global Catholicism. The Ashgate Research Companion to the Counter-Reformation presents a comprehensive examination of recent scholarship on early modern Catholicism in its many guises. It examines how the Tridentine reforms inspired conflict and conversion, and evaluates lives and identities, spirituality, culture and religious change. This wide-ranging and original research guide is a unique resource for scholars and students of European and transnational history

    Nonsynonymous mutations within APOB in human familial hypobetalipoproteinemia: evidence for feedback inhibition of lipogenesis and postendoplasmic reticulum degradation of apolipoprotein B.

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    Five nontruncating missense APOB mutations, namely A31P, G275S, L324M, G912D, and G945S, were identified in heterozygous carriers of familial hypobetalipoproteinemia (FHBL) in the Italian population. To test that the FHBL phenotype was a result of impaired hepatic secretion of mutant apoB proteins, we performed transfection studies using McA-RH7777 cells stably expressing wild type or mutant forms of human apolipoprotein B-48 (apoB-48). All mutant proteins displayed varied impairment in secretion, with G912D the least affected and A31P barely secreted. Although some A31P was degraded by proteasomes, a significant proportion of it (although inappropriately glycosylated) escaped endoplasmic reticulum (ER) quality control and presented in the Golgi compartment. Degradation of the post-ER A31P was achieved by autophagy. Expression of A31P also decreased secretion of endogenous apoB and triglycerides, yet the impaired lipoprotein secretion did not lead to lipid accumulation in the cells or ER stress. Rather, expression of genes involved in lipogenesis was down-regulated, including liver X receptor alpha, sterol regulator element-binding protein 1c, fatty acid synthase, acetyl-CoA carboxylase 1, stearoyl-CoA desaturase 1, and lipin-1. These results suggest that feedback inhibition of hepatic lipogenesis in conjunction with post-ER degradation of misfolded apoB proteins can contribute to reduce fat accumulation in the FHBL liver
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