Exploring contested authenticity among speakers of a contested language: the case of ‘Francoprovençal'

This paper explores the notion of speaker authenticity in the context of obsolescent ‘Francoprovençal’: a highly fragmented grouping of Romance varieties spoken in parts of France, Italy, and Switzerland by less than 1% of the total regional population. While Francoprovençal has long been losing ground to the dominant language(s) with which it is in contact, new speakers have begun to emerge within the context of revitalisation movements and activities geared more favourable language planning policies and increased literacy. The emergence of these new speakers has polarised native-speaker communities, and has blurred the lines associated with the traditional view of sociolinguistic authenticity. Through an analysis of qualitative data collected in 2012, this article argues in particular that it may not be sufficient to simply examine contested authenticities from a native–non-native perspective, but rather it is important to consider how new speakers might themselves form a complex spectrum of speaker types with new sets of tensions as has been argued elsewhere

A reference human induced pluripotent stem cell line for large-scale collaborative studies.

Human induced pluripotent stem cell (iPSC) lines are a powerful tool for studying development and disease, but the considerable phenotypic variation between lines makes it challenging to replicate key findings and integrate data across research groups. To address this issue, we sub-cloned candidate human iPSC lines and deeply characterized their genetic properties using whole genome sequencing, their genomic stability upon CRISPR-Cas9-based gene editing, and their phenotypic properties including differentiation to commonly used cell types. These studies identified KOLF2.1J as an all-around well-performing iPSC line. We then shared KOLF2.1J with groups around the world who tested its performance in head-to-head comparisons with their own preferred iPSC lines across a diverse range of differentiation protocols and functional assays. On the strength of these findings, we have made KOLF2.1J and its gene-edited derivative clones readily accessible to promote the standardization required for large-scale collaborative science in the stem cell field

Le Valaisan, tel qu'il est (langue et traditions populaires). Coutumes d'hier et coutumes d'aujourd'hui.

Schüle Ernest, Schüle Rose-C. Le Valaisan, tel qu'il est (langue et traditions populaires). Coutumes d'hier et coutumes d'aujourd'hui.. In: Le Globe. Revue genevoise de géographie, tome 100, 1960. pp. 12-13

Documents linguistiques de la Suisse romande I. Documents en langue française antérieurs à la fin du XIVe siècle conservés dans les cantons du Jura et de Berne

Documents linguistiques de la Suisse romande I. Documents en langue française antérieurs à la fin du XIVe siècle conservés dans les cantons du Jura et de Berne. Aubervilliers : Institut de Recherche et d'Histoire des Textes (IRHT), 2002. 716 p. (Documents, études et répertoires de l'Institut de Recherche et d'Histoire des Textes, 69

Identification of Cholestenoic acids that Regulate Motor Neuron Survival via Liver X Receptors

Cholestenoic acids are intermediates in metabolism of cholesterol to bile acids and their biosynthetic enzymes are expressed in the mammalian CNS. Here we describe the cholestenoic acid profile of mammalian cerebrospinal fluid and show that specific cholestenoic acids, including 3β,7α-dihydroxycholest-5-en-26-oic (3β,7α-diHCA) and 3β-hydroxy-7-oxocholest-5-en-26-oic acids (3βH,7O-CA), activate the liver X receptors (Lxrs), enhance Islet-1 expression and protein levels in zebrafish in vivo, and increase the number of oculomotor neurons in the developing mouse in vitro and in vivo. Mechanistically, while 3β,7α-diHCA promoted motor neuron survival in an Lxr-dependent manner, 3βH,7O-CA promoted maturation of precursors into Islet1+ cells. Surprisingly, a weak Lxr ligand, 3β-hydroxycholest-5-en-26-oic acid (3β-HCA), caused motor neuron cell loss in vivo. Mutations in CYP7B1 or CYP27A1, two enzymes involved in cholestenoic acid metabolism, result in two different neurological diseases hereditary spastic paresis type 5 (SPG5) and cerebrotendinous xanthomatosis (CTX), respectively. The former disease is characterized by spastic paresis and similar symptoms may sometimes occur also in some CTX patients. Analysis of CSF and plasma from patients with SPG5 revealed an excess of the toxic Lxr ligand, 3β-HCA, while patients with CTX and SPG5 exhibited low levels of the survival-promoting Lxr ligand, 3β,7α-diHCA in plasma. Combined, our results show that specific cholestenoic acids selectively work on motor neurons, via Lxr, to regulate the balance between survival and death. These findings suggest that efforts aimed at restoring the balance between toxic and pro-survival Lxr ligands, such as administration of 3β,7α-diHCA, can lead to potential treatments for motor neuron degeneration

Cholestenoic acids regulate motor neuron survival via liver X receptors

Cholestenoic acids are formed as intermediates in metabolism of cholesterol to bile acids, and the biosynthetic enzymes that generate cholestenoic acids are expressed in the mammalian CNS. Here, we evaluated the cholestenoic acid profile of mammalian cerebrospinal fluid (CSF) and determined that specific cholestenoic acids activate the liver X receptors (LXRs), enhance islet-1 expression in zebrafish, and increase the number of oculomotor neurons in the developing mouse in vitro and in vivo. While 3β,7α-dihydroxycholest-5-en-26-oic acid (3β,7α-diHCA) promoted motor neuron survival in an LXR-dependent manner, 3β-hydroxy-7-oxocholest-5-en-26-oic acid (3βH,7O-CA) promoted maturation of precursors into islet-1+ cells. Unlike 3β,7α-diHCA and 3βH,7O-CA, 3β-hydroxycholest-5-en-26-oic acid (3β-HCA) caused motor neuron cell loss in mice. Mutations in CYP7B1 or CYP27A1, which encode enzymes involved in cholestenoic acid metabolism, result in different neurological diseases, hereditary spastic paresis type 5 (SPG5) and cerebrotendinous xanthomatosis (CTX), respectively. SPG5 is characterized by spastic paresis, and similar symptoms may occur in CTX. Analysis of CSF and plasma from patients with SPG5 revealed an excess of the toxic LXR ligand, 3β-HCA, while patients with CTX and SPG5 exhibited low levels of the survival-promoting LXR ligand 3β,7α-diHCA. Moreover, 3β,7α-diHCA prevented the loss of motor neurons induced by 3β-HCA in the developing mouse midbrain in vivo.Our results indicate that specific cholestenoic acids selectively work on motor neurons, via LXR, to regulate the balance between survival and death