Hereditary gelsolin amyloidosis (HGA) is an autosomally dominantly inherited form of systemic amyloidosis, characterized mainly by cranial and sensory peripheral neuropathy, corneal lattice dystrophy, and cutis laxa. HGA, originally reported from Finland and now increasingly from other countries in Europe, North and South America, and Asia, may still be underdiagnosed worldwide. It is the first and so-far only known disorder caused by a gelsolin gene defect, namely a G654A or G654T mutation. Gelsolin is a principal actin-modulating protein, implicated in multiple biological processes, also in the nervous system, e.g. axonal transport, myelination, neurite outgrowth, and neuroprotection. The gelsolin gene defect causes expression of variant gelsolin, followed by systemic deposition of gelsolin amyloid (AGel) in HGA patients and even other consequences on the metabolism and function of gelsolin. In HGA, specific therapy is not yet available but correct diagnosis enables adequate symptomatic treatment which decisively improves the quality of life in these patients. A transgenic murine model of HGA expressing AGel is available, in anticipation of new treatment options targeted toward this slowly progressive but devastating amyloidosis. Present and future lessons learned from HGA may be applicable even in diagnosis and treatment of other hereditary and sporadic amyloidoses.
While deforestation continues at an alarming rate around the world, discussions on the range of underlying causes continue. The premise is that studying successful transitions from deforestation to sustainable forestry ex post in Finland can provide novel insights into how deforestation in the tropics might be reduced in the future. Our fundamental question here is why Finland succeeded to stop deforestation for a century ago and why not the same is feasible in the contemporary tropical countries? This book presents a novel integrated theory within which this case study on Finland and contemporary modeling of underlying causes of tropical deforestation are developed. Finland remains the world’s second largest net exporter of forest products, while maintaining the highest forest cover in Europe. A transition from deforestation to sustainable industrial forestry took place in Finland during the first part of the 20th century. The underlying causes of this transition are compared via our theory with deforestation in 74 contemporary tropical countries. Both appear similar and support our theory. The interaction of public policies and market institutions has appeared to be critical during this transition. The study’s findings suggest that private forest ownership with a continuous increase in the real value of forests and alleviation of poverty under non-corruptive conditions has been a necessary, but not a sufficient, condition for this transition. In a parallel way public policies have also proved to be a necessary, but not sufficient, condition in this transition. The conclusion is that socialistic forestry along with corruption is artificially maintaining too low values in the tropical forests. The opportunity cost of sustainable forestry remains too high and deforestation by extensification of agriculture therefore continues. The prevailing socialistic forestry with dominating public forest ownership is by purpose maintaining administratively set low stumpage prices leading to low value of forests, wide corruption and continuous forest degradation and deforestation. An effective remedy – to raise the value of forests - is found to be within forestry.
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