A proposal for engagement in MPAs in areas beyond national jurisdiction: The case of Macaronesia.

The Ocean is the largest ecosystem on the planet, supporting millions of people's livelihood. Marine protected areas (MPAs) are key to guarantee Ocean resilience. About 62 % of the Ocean surface coincide with areas 200 miles from coastlines, called areas beyond national jurisdiction (ABNJ). However, MPAs in ABNJ are only 1,18 % of the high seas. Stakeholder involvement is a keystone in the governance process. This is of particular importance in Macaronesia, and the need for compatibility between human activities and conservation, through the synergetic engagement of the local-maritime community. The present paper aims to develop a MPA proposal in the ABNJ context through expert consultation. The proposal was built upon the analysis of the Macaronesia region study case. Results show that there is no integrated international body to fully address the range of problems to be tackled, therefore the option to use the Conference of Parties model seems to be adequate. Considering Macaronesia region specific features, the conclusion is that select local focal points in each archipelago to enhance the local maritime community is essential. Furthermore, it would be necessary to implement Working Groups, rotative between archipelagos, to address different solutions for local conservation practices. Moreover, clear communication is fundamental since the very beginning to guarantee visibility and transparency.

Effect of endogenous purines on electrically evoked ACh release at the mouse neuromuscular junction.

At the mouse neuromuscular junction, adenosine triphosphate (ATP), which is co-released with the neurotransmitter acetylcholine (ACh), and its metabolite adenosine, modulate neurotransmitter release by activating presynaptic inhibitory P2Y₁₃ receptors (a subtype of ATP/adenosine diphosphate [ADP] receptor), inhibitory A₁ and A₃ adenosine receptors, and excitatory A_2A adenosine receptors. To study the effect of endogenous purines, when phrenic-diaphragm preparations are depolarized by different nerve stimulation patterns, we analyzed the effect of the antagonists for P2Y₁₃ , A₁ , A₃ , and A_2A receptors (AR-C69931MX, 8-cyclopentyl-1,3-dipropylxanthine, MRS-1191, and SCH-58261, respectively) on the amplitude of the end-plate potentials of the trains, and contrasted these results with those obtained with the selective agonists of these receptors (2-methylthioadenosine 5'-diphosphate trisodium salt hydrate, 2-chloro-N⁶ -cyclopentyl-adenosine, inosine, and PSB-0777, respectively). During continuous 0.5-Hz stimulation, the amount of endogenous purines was not enough to activate purinergic receptors, while at continuous 5-Hz stimulation, an incipient action of endogenous purines on P2Y₁₃ , A₁ and A3 receptors might be evident just at the end of the trains. During continuous 50-Hz stimulation, the concentration of endogenous ATP/ADP and adenosine exerted an inhibitory action on ACh release after of the initial phase of the train, but when the nerve was stimulated at intermittent 50 Hz (5 bursts), this behavior was not observed. Excitatory A_2A receptors were only activated when continuous 100-Hz stimulation was applied. In conclusion, when motor nerve terminals are depolarized by repetitive stimulation of the phrenic nerve, endogenous ATP/ADP and adenosine are able to fine-tune neurosecretion depending on the frequency and pattern of stimulation.

Allan-Herndon-Dudley syndrome and the monocarboxylate transporter 8 (MCT8) gene.

Allan-Herndon-Dudley syndrome was among the first of the X-linked mental retardation syndromes to be described (in 1944) and among the first to be regionally mapped on the X chromosome (in 1990). Six large families with the syndrome have been identified, and linkage studies have placed the gene locus in Xq13.2. Mutations in the monocarboxylate transporter 8 gene (MCT8) have been found in each of the six families. One essential function of the protein encoded by this gene appears to be the transport of triiodothyronine into neurons. Abnormal transporter function is reflected in elevated free triiodothyronine and lowered free thyroxine levels in the blood. Infancy and childhood in the Allan-Herndon-Dudley syndrome are marked by hypotonia, weakness, reduced muscle mass, and delay of developmental milestones. Facial manifestations are not distinctive, but the face tends to be elongated with bifrontal narrowing, and the ears are often simply formed or cupped. Some patients have myopathic facies. Generalized weakness is manifested by excessive drooling, forward positioning of the head and neck, failure to ambulate independently, or ataxia in those who do ambulate. Speech is dysarthric or absent altogether. Hypotonia gives way in adult life to spasticity. The hands exhibit dystonic and athetoid posturing and fisting. Cognitive development is severely impaired. No major malformations occur, intrauterine growth is not impaired, and head circumference and genital development are usually normal. Behavior tends to be passive, with little evidence of aggressive or disruptive behavior. Although clinical signs of thyroid dysfunction are usually absent in affected males, the disturbances in blood levels of thyroid hormones suggest the possibility of systematic detection through screening of high-risk populations.