Table 1.

Summary of prominent and overlapping protein mislocalization in cellular and animal laminopathy models.

Mislocalized Proteins	Role	Mislocalization	Model(s)/Patient Sample(s)
1. Lap2 (α, β)	regulation of nuclear architecture	absence in nuclear poles and/or lobules, honeycomb pattern in nuclear blebs	fibroblasts from homozygous p.Y259X patient [46], Lmna LAO mice [109], LmnaΔ8–11 mice MEFs [61]
2. Emerin	anchorage to the cytoskeleton	cytoplasmic, concentration in one pole, sequestration within nuclear lamin foci, honeycomb pattern	fibroblasts from homozygous p.Y259X patient [46], RNAi LMNA knockdown in HeLa cells [48], various lamin A/C variants expressed in different cell lines [49,50], C. elegans [90], fibroblasts from patients with various lamin A/C mutations [51], myogenic cells derived from myopathies patients’ iPSCs [81], LmnaΔ8–11 mice [50,61], LmnaN195K/N195K mice [86], p.K46del lamin-1 variant C. elegans [87]
3. Syne1	anchorage to the cytoskeleton	cytoplasmic	fibroblasts from homozygous p.Y259X patient [46]
4. B-type lamins	involved in a variety of functions including regulation of expression, mitosis, cellular senescence	absence in one pole (i.e., concentration in one pole only) and/or absence in nuclear lobules, honeycomb pattern	fibroblasts from homozygous p.Y259X patient [46], fibroblasts from patients with various lamin A/C mutations [51], myogenic cells derived from myopathies patients’ iPSCs [81], LamC null and Lamin-C N-terminal deleted D. melanogaster mutants [93], LmnaΔ8–11 mice MEFs [61], cardiac expressing p.M371K lamin A/C mice [119]
5. Nup153, Nup154	component of the nuclear pore complex	absence in nuclear poles and/or lobules, clustering	fibroblasts from homozygous p.Y259X patient [46], fibroblasts from p. R225X patient [79], RNAi lmn-1 knockdown in C. elegans [83], LmnaΔ8–11 mice [61], LmnaN195K/N195K mice [86], LamC null and various lamin A/C mutations expressed in D. melanogaster [96], LmnaGT−/− mice [103], LmnaΔ8–11 mice MEFs [61]
6. SUMO1	post-translational modifications	sequestration within nuclear lamin foci	various lamin A/C variants expressed in Cos7 cells [38], C2C12 cells [78], LmnaH222P/H222P mice primary myoblasts [78], LmnaH222P/H222P mice skeletal muscle tissue [78]
7. Actin	cytoskeletal component	filament disorganization, increased nuclear localization, and decreased expression	neonatal rat ventricular myocytes expressing various mutant lamin A/C [34], LamC null D. melanogaster [96], Lamin-C N-terminal deleted and various lamin A/C mutations expressed in D. melanogaster [97], patient myoblasts expressing various L-CMD variants [35,36]
8. ERK ½ (phosphorylated)	involved in a variety of cellular responses	increased nuclear localization	LmnaH222P/H222P mice, p.H222P lamin A expressing Cos7 and C2C12 cells [21]
9. Smad2/3 (phosphorylated)	TGF-β signalling pathway	increased nuclear localization	LmnaH222P/H222P mice [118]
10. Androgen receptors, SRF -FHL2	mediating actions of androgens	nuclear accumulation	neonatal rat cardiomyocytes expressing p.H222P variant or p.R225X variant [121], LmnaH222P/H222P mice and cardiac tissue from DCM patients [121]
11. Cx40, Cx43	gap junction proteins	Diffused pattern and decreased expression in atria	LmnaN195K/N195K mice [86]