. 2019 Mar 29;8(4):291. doi: 10.3390/cells8040291

Table 2.

Summary of prominent and overlapping affected proteins and signalling pathways in cellular and animal laminopathy models.

Models		Affected Proteins and Signalling Pathways	Reference(s)
Cellular Models	1. Fibroblasts from p.E203K DCM patient or HeLa transfected cells	↓ sumoylation	[77]
	2. Various lamin A/C variants expressed in neonatal rat ventricular myocytes	inhibition of p38 = rescue of actin and mechanical phenotype	[34]
	3. p. L530P (EDMD)	↓ binding to SREBF1	[15]
	4. Fibroblasts from p. R225X patient	inhibition of MEK/ERK 1/2 = rescue (decreased apoptosis and senescence)	[79]
	5. Myoblasts from patients expressing various L-CMD variants	deregulation of yes-associated protein and formins	[35,36]
Animal Models	6. Various lamin A/C variants expressed in D. melanogaster	↑ Nrf2, p62/SQSTM1	[97]
	7. D. rerio lmna morphants	↓ pparγ, ↓ PCNA	[101]
	8. D. rerio lmna transgenics	↑ PCNA	[102]
	9. Lmna^nPLAO/nPLAO mice	↓ Myh6, ↑ Myh7	[108]
	10. Lmna^N195K/N195K mice	reactivation of foetal genes (↑ANP, ↑ BNP), inhibition of Na⁺ channel = improve symptom, ↓ connexin 40, mis-expression of Hf1b/Sp4	[86]
	11. Mice cardiac tissue only expression of p.E82K lamin A/C variant	hypertrophy markers (↑ in BNP, actin alpha 1, collagen type III alpha 1), ↑ FAS, ↑ cytochrome C	[115]
	12. Lmna^H222P/H222P mice	TGFβ (↑ nuclear phos-Smad 2/3), ↑ MAPK members (ERK 1/2, p38, JNK),↑ AKT/mTor, ↓ WNT/β-catenin	[21,67,121,122,123,124,125,126,127,128,129]
	13. Lmna^ΔK32/ΔK32 mice	repression of SREBF1	[130]
	14. Muscle tissues from patients with various myopathies	phos-lamin A/C (Ser458) by Akt	[73]
	15. Lmna^ΔK32/+ mice	↑ Nppb, ↑ Myh7	[65]