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000257582 1001_ $$0P:(DE-2719)9000667$$aMesselodi, Daria$$b0$$udzne
000257582 245__ $$aNeuronopathic Gaucher disease models reveal defects in cell growth promoted by Hippo pathway activation.
000257582 260__ $$aLondon$$bSpringer Nature$$c2023
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000257582 520__ $$aGaucher Disease (GD), the most common lysosomal disorder, arises from mutations in the GBA1 gene and is characterized by a wide spectrum of phenotypes, ranging from mild hematological and visceral involvement to severe neurological disease. Neuronopathic patients display dramatic neuronal loss and increased neuroinflammation, whose molecular basis are still unclear. Using a combination of Drosophila dGBA1b loss-of-function models and GD patient-derived iPSCs differentiated towards neuronal precursors and mature neurons we showed that different GD- tissues and neuronal cells display an impairment of growth mechanisms with an increased cell death and reduced proliferation. These phenotypes are coupled with the downregulation of several Hippo transcriptional targets, mainly involved in cells and tissue growth, and YAP exclusion from nuclei. Interestingly, Hippo knock-down in the GBA-KO flies rescues the proliferative defect, suggesting that targeting the Hippo pathway can be a promising therapeutic approach to neuronopathic GD.
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000257582 650_7 $$0EC 3.2.1.45$$2NLM Chemicals$$aGlucosylceramidase
000257582 650_2 $$2MeSH$$aHumans
000257582 650_2 $$2MeSH$$aGaucher Disease: genetics
000257582 650_2 $$2MeSH$$aGaucher Disease: metabolism
000257582 650_2 $$2MeSH$$aGaucher Disease: therapy
000257582 650_2 $$2MeSH$$aGlucosylceramidase: genetics
000257582 650_2 $$2MeSH$$aGlucosylceramidase: metabolism
000257582 650_2 $$2MeSH$$aHippo Signaling Pathway
000257582 650_2 $$2MeSH$$aNeurons: metabolism
000257582 650_2 $$2MeSH$$aCell Proliferation
000257582 7001_ $$00000-0003-1248-4498$$aStrocchi, Silvia$$b1
000257582 7001_ $$aBertuccio, Salvatore Nicola$$b2
000257582 7001_ $$0P:(DE-2719)2811884$$aBaden, Pascale$$b3$$udzne
000257582 7001_ $$aIndio, Valentina$$b4
000257582 7001_ $$00000-0002-7325-9908$$aGiorgi, Federico M$$b5
000257582 7001_ $$aTaddia, Alberto$$b6
000257582 7001_ $$aSerravalle, Salvatore$$b7
000257582 7001_ $$aValente, Sabrina$$b8
000257582 7001_ $$00000-0001-6478-026X$$adi Fonzo, Alessio$$b9
000257582 7001_ $$aFrattini, Emanuele$$b10
000257582 7001_ $$aBernardoni, Roberto$$b11
000257582 7001_ $$aPession, Annalisa$$b12
000257582 7001_ $$00000-0001-7429-4062$$aGrifoni, Daniela$$b13
000257582 7001_ $$0P:(DE-2719)2810385$$aDeleidi, Michela$$b14
000257582 7001_ $$00000-0002-2732-0747$$aAstolfi, Annalisa$$b15
000257582 7001_ $$aPession, Andrea$$b16
000257582 773__ $$0PERI:(DE-600)2919698-X$$a10.1038/s42003-023-04813-2$$gVol. 6, no. 1, p. 431$$n1$$p431$$tCommunications biology$$v6$$x2399-3642$$y2023
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