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a slight but significant point of contention in this field is the attribution of the clinical fdt phenotype to progranulin. so far, researchers have found that this gene, like app, causes pathology in mouse models. but with only one previous study that suggested pglua as a second hit in mice, several of the authors of earlier studies have come under scrutiny for assigning clinical fdt to the gene rather than to the complex that includes pglua. however, all recent work makes clear that the link between progranulin and fdt is real. the current view is that progranulin causes the fdt in ad patients, for similar reasons as it causes the dementia: either by exerting a toxic effect on neurons or by failing to support neuron health. in this news series, rademakers' colleague birgit de strooper discussed data suggesting that progranulin loss of function is a risk factor for the early, pre-symptomatic stage of ad when pathology starts and these neurons begin to lose their mitochondrial functions ( de strooper et al., 2007 ). it still seems premature to think of progranulin as an available therapy, but it is likely that its importance in this area will become even more clear as the field moves forward.

in lisbon, the mutant elavl4 protein, known as hud, is a neuron-expressed rna-binding protein essential for the proper development and function of the brain. antónio carrapico, the president of the elavl4 research consortium, studied the function of hud as a possible therapeutic target for amyotrophic lateral sclerosis (als). to this end, carrapico prepared a “knockout” of the mouse gene hud as the mouse model for the disease. this model shows morphological alterations resembling those seen in als patients, including vacuolization of cortical and spinal motor neurons. although the mouse model does not represent als in all its features, data collected in mouse organotypic spinal cultures (df) and in primary rodent spinal cultures ( df) reveal that the rna-binding protein hud plays a critical role in motor neuron development. this model can be useful to identify the therapeutic mechanisms with hud or other targets in als, for the development of therapeutic strategies targeting hud and further to identify therapeutic strategies for als. from his collaborative group in spain, matías sanz presented data showing the function of another rna-binding protein, tdp43, in the nervous system. this group reported that tdp43 is mutated in als patients, causing the protein to misfold and form aggregates called ubiquitinated inclusions that contain proteolytically processed tdp43. a mutant form of tdp43 functions normally, but crosses the cellular membrane and accumulates in the cytosol of motor neurons. this mutation of tdp43 probably disrupts its normal function and causes motor neuron death. this information supports the idea that tdp43 is a central protein in the pathogenesis of als and suggests that inhibition of tdp43 accumulation is a promising therapeutic strategy for als. 84d34552a1