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000281808 0247_ $$2doi$$a10.1007/s00018-025-05898-0
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000281808 1001_ $$00000-0003-3445-2753$$aMenge, Sonja$$b0
000281808 245__ $$aComparing loss of individual fragile X proteins suggests strong links to cellular senescence and aging.
000281808 260__ $$aCham (ZG)$$bSpringer International Publishing AG$$c2025
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000281808 520__ $$aMembers of the fragile X protein (FXP) family (FMR1, FXR1 and FXR2) are differentially expressed in most types of cancer and major neurodegenerative diseases. While increased expression of FXR1 in cancer has been linked to senescence evasion and consequently tumor initiation and progression, decreased expression of FXPs in neurodegeneration may contribute to pathogenic protein aggregation and death of vulnerable neurons. However, due the causal role in fragile x syndrome, most data are available about loss of FMR1 in neurons while functions of FXR1 and especially FXR2 remain largely unexplored. To address this knowledge gap, and to directly compare functions of the FXPs, we used proteomics of CRISPR/Cas9 edited HAP1 cells carrying knockouts of the individual FXPs for identification of cellular mechanisms associated with these proteins. Further exploration of proteomic findings suggests roles of the FXPs in ribosome biogenesis, autophagy and mitochondrial health linked to organismal aging, and cellular senescence. Validation of FXP induced defects relevant for neurodegenerative diseases in neuroblastoma cell line SH-SY5Y upon FXP knockdown revealed high cell type specificity of individual FXP functions. Overall, we provide a comprehensive overview and comparison of cellular mechanisms related to the individual FXPs, as well as starting points for further studying this protein family in respective cell types of FXP associated diseases, and in aging in general.
000281808 536__ $$0G:(DE-HGF)POF4-352$$a352 - Disease Mechanisms (POF4-352)$$cPOF4-352$$fPOF IV$$x0
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000281808 650_7 $$2Other$$aAlzheimer’s disease
000281808 650_7 $$2Other$$aAmyotrophic lateral sclerosis
000281808 650_7 $$2Other$$aFragile x syndrome
000281808 650_7 $$2Other$$aParkinson’s disease
000281808 650_7 $$2Other$$aProtein aggregation
000281808 650_7 $$0139135-51-6$$2NLM Chemicals$$aFragile X Mental Retardation Protein
000281808 650_7 $$2NLM Chemicals$$aRNA-Binding Proteins
000281808 650_7 $$2NLM Chemicals$$aFMR1 protein, human
000281808 650_7 $$2NLM Chemicals$$aFXR1 protein, human
000281808 650_2 $$2MeSH$$aHumans
000281808 650_2 $$2MeSH$$aCellular Senescence: genetics
000281808 650_2 $$2MeSH$$aFragile X Mental Retardation Protein: genetics
000281808 650_2 $$2MeSH$$aFragile X Mental Retardation Protein: metabolism
000281808 650_2 $$2MeSH$$aAging: genetics
000281808 650_2 $$2MeSH$$aAging: metabolism
000281808 650_2 $$2MeSH$$aRNA-Binding Proteins: metabolism
000281808 650_2 $$2MeSH$$aRNA-Binding Proteins: genetics
000281808 650_2 $$2MeSH$$aCRISPR-Cas Systems
000281808 650_2 $$2MeSH$$aAutophagy
000281808 650_2 $$2MeSH$$aCell Line, Tumor
000281808 650_2 $$2MeSH$$aProteomics: methods
000281808 650_2 $$2MeSH$$aMitochondria: metabolism
000281808 650_2 $$2MeSH$$aFragile X Syndrome: metabolism
000281808 650_2 $$2MeSH$$aFragile X Syndrome: genetics
000281808 650_2 $$2MeSH$$aFragile X Syndrome: pathology
000281808 650_2 $$2MeSH$$aNeurodegenerative Diseases: metabolism
000281808 650_2 $$2MeSH$$aNeurodegenerative Diseases: genetics
000281808 650_2 $$2MeSH$$aNeurodegenerative Diseases: pathology
000281808 7001_ $$00000-0003-1849-5569$$aSegura, Inmaculada$$b1
000281808 7001_ $$aHartmann, Max$$b2
000281808 7001_ $$aDecker, Lorena$$b3
000281808 7001_ $$0P:(DE-2719)9001513$$aKiran, Selin$$b4
000281808 7001_ $$0P:(DE-2719)9001513$$aDanzer, Karin M$$b5$$udzne
000281808 7001_ $$aIben, Sebastian$$b6
000281808 7001_ $$00000-0002-4568-9003$$aHarbauer, Angelika B$$b7
000281808 7001_ $$0P:(DE-2719)9001560$$aOeckl, Patrick$$b8
000281808 7001_ $$0P:(DE-2719)9001054$$aFreischmidt, Axel$$b9
000281808 773__ $$0PERI:(DE-600)1458497-9$$a10.1007/s00018-025-05898-0$$gVol. 82, no. 1, p. 358$$n1$$p358$$tCellular and molecular life sciences$$v82$$x1420-682X$$y2025
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