Table 2.

Nups and other nucleocytoplasmic transport proteins in premature aging and neurodegeneration.

Disease	Pathogenic macromolecules of interest	Experimental systems (excluding non-mammalian models)	Mislocalized or coaggregated proteins	Reference
HGPS	Progerin	Patient fibroblasts	Tpr and Ran	Kelley et al., 2011
			Nup153 and Transportin-1	Larrieu et al., 2018
ALS	N/A	iPSC-derived neurons and patient brains	Nup107, Nup205, Ran, and RanGAP1	Zhang et al., 2015
	N/A	iPSC-derived neurons	RCC1	Jovicic et al., 2015
	(GR)n and (PR)n	HEK293T	Nup155, Nup205, Ran, RanGAP1, and several karyopherins	Lee et al., 2016
	(GA)n	Mouse models	Pom121 and RanGAP1	Zhang et al., 2016
	(PR)n	Recombinant proteins	Nup98 and Nup54	Shi et al., 2017
	Arginine-containing DPRs	HEK293T lysate	>20 Nups and transport factors	Hayes et al., 2020
	(G4C2)n and (G2G4)n RNAs	iPSC-derived neurons and patient brains	Pom121, Ndc1, Nup210, Tpr, Nup50, Nup98, Nup107,and Nup133	Coyne et al., 2020
	TDP-43	Neuro-2a cell line and patient brains	>20 Nups and transport factors	Chou et al., 2018
Huntington’s	Polyglutamine- expanded	Mouse models, iPSC-derived neurons, and patient brains	Gle1 and RanGAP1	Gasset-Rosa et al., 2017
	HTT	Mouse models, iPSC-derived neurons, and patient brains	Nup62, Nup88, Ran, and RanGAP1	Grima et al., 2017
Alzheimer’s	Tau	Mouse models and patient brains	Nup98 and Ran	Eftekharzadeh et al., 2018