Dysregulation of ubiquitin homeostasis and β-catenin signaling promote spinal muscular atrophy.

The autosomal recessive neurodegenerative disease spinal muscular atrophy (SMA) results from low levels of survival motor neuron (SMN) protein; however, it is unclear how reduced SMN promotes SMA development. Here, we determined that ubiquitin-dependent pathways regulate neuromuscular pathology in SMA. Using mouse models of SMA, we observed widespread perturbations in ubiquitin homeostasis, including reduced levels of ubiquitin-like modifier activating enzyme 1 (UBA1).

Immunotoxicity and oxidative stress in the Arctic scallop Chlamys islandica: effects of acute oil exposure.

With increasing oil exploration in Arctic regions, the risk of an accidental oil spill into the environment is inevitably elevated. As a result, concerns have been raised over the potential impact of oil exposure on Arctic organisms. This study assessed the effects of an acute oil exposure (mimicking an accidental spill) on the immune function and oxidative stress status of the Arctic scallop Chlamys islandica.

Effects of the model PAH phenanthrene on immune function and oxidative stress in the haemolymph of the temperate scallop Pecten maximus.

Phenanthrene, a major component of crude oil, is one of the most abundant PAHs in aquatic ecosystems, and is readily bioavailable and toxic to a range of marine invertebrates. Within bivalves, the haemolymph acts as a transfer medium for these pollutants and their metabolic products, leaving haemocytes susceptible to deleterious effects. Using a suite of biological endpoints, this study determined the sublethal (7-d exposure to 50, 100 and 200microgL(-1)) effects of phenanthrene on several oxidative stress and immunological parameters in the haemolymph of the commercially-important scallop Pecten maximus.

Functional immune response in Pecten maximus: combined effects of a pathogen-associated molecular pattern and PAH exposure.

Pathogen-associated molecular patterns (PAMPs) enable recognition of structures present in microorganisms such as lipopolysaccharides (LPS). LPS are an essential constituent of the outer membrane of Gram-negative bacteria, stimulating the innate immune system of invertebrates. Here, LPS from Escherichia coli (055:B5) were used to investigate the functional immune response of Pecten maximus after stimulation with a PAMP and to determine the combined effect of a phenanthrene exposure and LPS challenge.

Immune function in the Arctic Scallop, Chlamys islandica, following dispersed oil exposure.

With the current expansion of offshore oil activities in Arctic regions, there is an urgent need to establish the potential effects of oil-related compounds on Arctic organisms. As susceptibility to growth, disease and survival is determined partly by the condition of an organism's immune system, measurement of endpoints linked to the latter system provide important early warning signals of the sub-lethal effects of exposure to contaminants. This study assessed the impact of dispersed oil exposure on immune endpoints in the Arctic Scallop Chlamys islandica, using a combination of cellular and humoral biological responses.

Characterisation of esterases as potential biomarkers of pesticide exposure in the lugworm Arenicola marina (Annelida: Polychaeta).

Here, we identify and characterise cholinesterase (ChE) and carboxylesterase (CbE) activities in the body tissues of the sediment dwelling worm Arenicola marina. Exposure to the organophosphorus pesticide azamethiphos yielded an in vitro IC50 of 5 microg l(-1) for propionylcholinesterase (PChE). PChE was significantly inhibited in vivo after a 10 day exposure to 100 microg l(-1) azamethiphos, equivalent to the recommended aquatic application rate (ANOVA; F=2.