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000280968 0247_ $$2doi$$a10.1016/j.celrep.2025.116113
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000280968 041__ $$aEnglish
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000280968 1001_ $$0P:(DE-2719)9000982$$aSzewczyk, Barbara$$b0$$udzne
000280968 245__ $$aActivation of polo-like kinase 1 correlates with selective motor neuron vulnerability in familial ALS.
000280968 260__ $$aMaryland Heights, MO$$bCell Press$$c2025
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000280968 520__ $$aMutations in the Fused in Sarcoma (FUS) gene cause familial amyotrophic lateral sclerosis (ALS), characterized by selective degeneration of spinal motor neurons (sMNs) with relative sparing of cortical neurons (CNs). The mechanisms underlying this cell-type vulnerability remain unclear. Here, we compare CNs and sMNs derived from FUS-ALS models to assess differential responses to FUS mutations. We find that CNs are less affected than sMNs in DNA damage repair, axonal organelle trafficking, and stress granule dynamics. RNA sequencing (RNA-seq) reveals distinct transcriptomic signatures, with sMNs uniquely activating DNA damage responses involving cell cycle regulators, particularly polo-like kinase 1 (PLK1). PLK1 is highly expressed in sMNs but not CNs, correlating with greater nuclear FUS loss and splicing defects in sMNs. Cross-comparison with other familial ALS RNA-seq datasets highlights PLK1 upregulation as a shared molecular feature. These findings identify intrinsic differences between CNs and sMNs in FUS-ALS and suggest PLK1 as a potential driver of sMN vulnerability.
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000280968 650_7 $$2Other$$aCP: Molecular biology
000280968 650_7 $$2Other$$aCP: Neuroscience
000280968 650_7 $$2Other$$aDNA damage response
000280968 650_7 $$2Other$$aFUS loss of function
000280968 650_7 $$2Other$$aFUS-ALS
000280968 650_7 $$2Other$$aPLK1
000280968 650_7 $$2Other$$aneurodegeneration
000280968 650_7 $$2Other$$apolo-like kinase 1
000280968 650_7 $$2Other$$aselective vulnerability
000280968 650_7 $$2Other$$atranscriptomics
000280968 7001_ $$aZimyanin, Vitaly$$b1
000280968 7001_ $$aJaptok, Julia$$b2
000280968 7001_ $$aHeld, Aaron$$b3
000280968 7001_ $$aPal, Arun$$b4
000280968 7001_ $$aGroßmann, Dajana$$b5
000280968 7001_ $$aGlaß, Hannes$$b6
000280968 7001_ $$aJürs, Alexandra V$$b7
000280968 7001_ $$aDash, Banaja P$$b8
000280968 7001_ $$aBak, Maciek$$b9
000280968 7001_ $$aNaumann, Marcel$$b10
000280968 7001_ $$aHartmann, Christiane$$b11
000280968 7001_ $$aKuksenko, Olena$$b12
000280968 7001_ $$0P:(DE-2719)2811849$$aGünther, René$$b13$$udzne
000280968 7001_ $$aKao, Tzu-Ting$$b14
000280968 7001_ $$aSameith, Katrin$$b15
000280968 7001_ $$aDahl, Andreas$$b16
000280968 7001_ $$aSterneckert, Jared$$b17
000280968 7001_ $$aAronica, Eleonora$$b18
000280968 7001_ $$aShneider, Neil A$$b19
000280968 7001_ $$aBüttner, Andreas$$b20
000280968 7001_ $$0P:(DE-2719)9001873$$aCatanese, Alberto$$b21$$udzne
000280968 7001_ $$aPhatnani, Hemali$$b22
000280968 7001_ $$0P:(DE-HGF)0$$aKipp, Markus$$b23
000280968 7001_ $$aWainger, Brian J$$b24
000280968 7001_ $$aGoswami, Anand$$b25
000280968 7001_ $$0P:(DE-2719)2811732$$aHermann, Andreas$$b26$$eLast author$$udzne
000280968 773__ $$0PERI:(DE-600)2649101-1$$a10.1016/j.celrep.2025.116113$$gVol. 44, no. 9, p. 116113 -$$n9$$p116113$$tCell reports$$v44$$x2211-1247$$y2025
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