Mitochondrial dysfunction and Purkinje cell loss in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS).

Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a childhood-onset neurological disease resulting from mutations in the SACS gene encoding sacsin, a 4,579-aa protein of unknown function. Originally identified as a founder disease in Québec, ARSACS is now recognized worldwide. Prominent features include pyramidal spasticity and cerebellar ataxia, but the underlying pathology and pathophysiological mechanisms are unknown.

The clavesin family, neuron-specific lipid- and clathrin-binding Sec14 proteins regulating lysosomal morphology.

Clathrin-coated vesicles (CCVs) originating from the trans-Golgi network (TGN) provide a major transport pathway from the secretory system to endosomes/lysosomes. Herein we describe paralogous Sec14 domain-bearing proteins, clavesin 1/CRALBPL and clavesin 2, identified through a proteomic analysis of CCVs. Clavesins are enriched on CCVs and form a complex with clathrin heavy chain (CHC) and adaptor protein-1, major coat components of TGN-derived CCVs.

Direct protein detection with a nano-interdigitated array gate MOSFET.

A new protein sensor is demonstrated by replacing the gate of a metal oxide semiconductor field effect transistor (MOSFET) with a nano-interdigitated array (nIDA). The sensor is able to detect the binding reaction of a typical antibody Ixodes ricinus immunosuppressor (anti-Iris) protein at a concentration lower than 1 ng/ml. The sensor exhibits a high selectivity and reproducible specific detection.

Synthesis and palladium-catalysed isomerisation of fused polycyclic tetrahydrofurans: efficient and stereoselective one-pot domino construction of functionalised bridged bicyclo[n.2.1] ring systems.

A new one-pot domino reaction for a general entry to functionalised bridged bicyclo[n.2.1] ring systems from alpha, alpha'-diactivated cyclic ketones and trans-1,4-dihalides is described.

Farm diversity, classification schemes and multifunctionality.

Diversity is not only intrinsic to agriculture; it can be considered also as one of its main assets as it provides a wide range of responses that can help to face uncertain futures. The ongoing encounter between changing spatial and temporal frameworks and a set of diverse farming strategies is leading to the emergence of an ongoing flow of development models that could materialize in a wide range of farming practices, contrasting enterprise models, changing relations between rural households and agricultural holdings, and differentiated patterns that link farming and farming families to the wider context in which they are embedded. The many-sided diversity encountered in agriculture is not only the outcome of the agency and polyvalence of the actors involved; their agency and polyvalence are in turn inspired and strengthened by the material and symbolic diversity, which contributes to a further unfolding of diversity.