Recombinant Mouse Prelamin-A/C (Lmna)

1. Data show that lamin A/C expressing cells can form an actin cap to resist nuclear deformation in response to physiological mechanical stresses. PMID: 29242553
2. THE ROLE OF LMNA MUTATIONS IN MYOGENIC DIFFERENTIATION OF PRIMARY SATELLITE CELLS AND C2C12 CELLS. PMID: 30199159
3. Using the thin film method combined with sample specific finite element modeling, we quantitatively showed a fivefold lower contractile stress generation of Lmna knockout embryonic fibroblasts (MEFs) as compared to wild-type MEFs. Via fluorescence microscopy it was demonstrated that the lower contractile stress generation was associated with an impaired actin stress fiber organization with thinner actin fibers. PMID: 28702670
4. Lmna gene product might play a crucial role in the ChAT-dependent molecular differentiation cascade. PMID: 27221609
5. Heterozygous Lmna(delK32/+) (Het) mice had similar cardiac function to wild-type (Wt) before exercise; after 5 weeks strenuous running, Het mice showed cardiac dysfunction and dilation without visible changes in cardiac morphology, molecular remodelling or nuclear structure compared to Wt exercised and Het sedentary mice. Skeletal muscle ex vivo contractile function remained unaffected in Het exercised mice. PMID: 27287550
6. Alterations in ventricular myocytes ion channel properties may contribute to arrhythmia and sudden cardiac death in LmnaN195K mutation-related Familial Dilated Cardiomyopathy. PMID: 27498076
7. our findings indicate that altered mTOR signaling in Lmna(-/-) mice leads to a lipodystrophic phenotype that can be rescued with rapamycin, highlighting the effect of loss of adipose tissue in Lmna(-/-) mice and the consequences of altered mTOR signaling PMID: 27926859
8. results suggest that lamin A plays important roles in maintaining the osteoblast differentiation and function PMID: 28237702
9. these findings show that cardiac ERK1/2 activity is modulated in part by TGF-b/Smad signaling, leading to altered activation of CTGF/CCN2 to mediate fibrosis and alter cardiac function. This identifies a novel mechanism in the development of LMNA cardiomyopathy. PMID: 27131347
10. Activation of WNT/b-catenin activity improved cardiac contractility and ameliorated intraventricular conduction defects in LmnaH222P/H222P mice, which was associated with increased expression of myocardial connexin 43. These results indicate that decreased WNT/b-catenin contributes to the pathophysiology of LMNA cardiomyopathy and that drugs activating b-catenin may be beneficial in affected individuals PMID: 28069793
11. SUMO1 conjugation of RB and Lamin A/C is modulated by the SUMO protease SENP1 and that sumoylation of both proteins is required for their interaction. PMID: 27270425
12. Lmna-deficient cells show a compromised strain avoidance response, which is completely abolished when topographical cues and uniaxial strain are applied along the same direction. PMID: 28062850
13. While the distribution patterns of both lamins closely paralleled the respective stages of mitosis, Nup160 localization in metaphase oocytes corresponded to that in mitotic prometaphase rather than metaphase. PMID: 28374669
14. changes in nuclear size and shape, which are mediated by nuclear envelope structural proteins lamin A/C and/or emerin, also impact gene regulation and lineage differentiation in early embryos. PMID: 28088180
15. specific laminopathy-associated mutations exhibit both positive and negative effects on prelamin A accumulation, indicating that these mutations affect prelamin A processing efficiency in different manners. PMID: 26900797
16. administration of the exon 11 ASO reduced lamin A expression in wild-type mice and progerin expression in an HGPS mouse model. PMID: 26999604
17. stabilization of perinuclear actin strengthens the transient interactions of lamin A with chromatin PMID: 26359759
18. It was found that the lamin A protein expressed in mouse ear cartilage cells is shorter than protein expressed in mouse skin. This difference in protein length could be caused by differential cleavage in the cells of skin and ear cartilage tissues PMID: 26204409
19. Annexin, lamin, and vimentin were identified as universal dystrophic markers PMID: 26102067
20. These results identify SNX6 as a key regulator of lamin A synthesis and incorporation into the nuclear envelope. PMID: 25535984
21. Progerin and full-length farnesyl-prelamin A are toxic to neurons of the enteric nervous system. PMID: 25652409
22. loss of Lmna under malignant conditions may, to a limited extent, enhance polyp size indicating that A-type lamins may regulate cell proliferation in the transformed GI epithelium PMID: 25578479
23. The results presented in this work indicate that the detrimental effects of lamin A expression were less than anticipated in the brain, even after 90 weeks of transgenic expression. PMID: 25343989
24. ablation of the kinase CDK2 alters the nuclear envelope in mouse spermatocytes, and that the proteins SUN1, KASH5 (also known as CCDC155) and lamin C2 show an abnormal cap-like distribution facing the centrosome. PMID: 25380821
25. Results suggest that lamin A/C is critical for proper structural and functional development of the sperm acrosome and head shape. PMID: 25118303
26. progerin and p16(INK4a) expression, beta-galactosidase activity, and reactive oxygen species, which increase with age, were higher in young Xpc(-/-) mice than in young Xpc(+/+) ones PMID: 25437426
27. In mouse fibroblasts directed targeting of chromatin to the nuclear lamina is mediated by chromatin state and A-type lamins. PMID: 25559185
28. only lamin-A can ensure the localization of emerin within the nuclear lamina PMID: 24523288
29. These findings underscore the importance of A-type lamins for TCR activation and identify lamin-A as a previously unappreciated regulator of the immune response. PMID: 24757177
30. Both ACE inhibition and MEK1/2 inhibition have beneficial effects on left ventricular function in Lmna(H222P/H222P) mice and both drugs together have a synergistic benefit when initiated after the onset of left ventricular dysfunction. PMID: 25218145
31. Studies demonstrate the in vivo importance of prelamin A's 3' UTR and its miR-9 binding site in regulating lamin A expression in the brain. PMID: 24203701
32. Overexpression of the most common progeroid lamin A mutation (LMNA c.1824C>T, p.G608G) during skin development results in a severe phenotype, characterized by dry scaly skin. PMID: 24305605
33. Lamin A Deltaexon9 mutation leads to telomere and chromatin defects PMID: 24153156
34. Report age-related changes in LMNA splicing and expression of progerin in mouse skeletal muscles. PMID: 24294364
35. Genetic evidence shows that ERK1 and ERK2 contribute to the development of cardiomyopathy caused by LMNA mutations and reveal interplay between these isoenzymes in maintaining a combined pathological activity in heart. PMID: 23933734
36. Lifespan and body mass were increased in Lmna(-/-)Lap2alpha(-/-) mice compared with Lmna deficient mice. PMID: 23535822
37. Homozygous LmnaH222P/H222P mice demonstrated a nuclear accumulation of androgen receptor and showed gender differences. PMID: 23631840
38. prelamin A accumulation does not impair tumour initiation and growth, but it decreases the incidence of infiltrating oral carcinomas. PMID: 23917225
39. Heterozygous LmnadelK32 mice develop dilated cardiomyopathy through a combined pathomechanism of haploinsufficiency and peptide toxicity. PMID: 23575224
40. Significant effects on embryonic stem cell differentiation to visceral endoderm, neuronal and myogenic lineages upon depletion of lamin A/C. PMID: 23451281
41. Lmna homozygous mutants (Lmna(Delta/Delta)) display postnatal lethality at postnatal 16-18 days. PMID: 23998933
42. Data indicate that a LAP2alpha-independent assembly defect of DeltaK32 lamin A/C is the cause of the pathology, whereas the LAP2alpha-linked functions of nucleoplasmic lamin A/C in the regulation of tissue progenitor cells are not affected in Lmna(DeltaK32/DeltaK32) mice. PMID: 23444379
43. Tissue stiffness and stress thus increase lamin-A levels, which stabilize the nucleus while also contributing to lineage determination. PMID: 23990565
44. a novel mechanism that could provide insight into the disease aetiology for the cardiac phenotype in many laminopathies, whereby lamin A/C and emerin regulate gene expression through modulation of nuclear and cytoskeletal actin polymerization PMID: 23644458
45. Akt phosphorylation at S404 targets the precursor prelamin A for degradation. Akt also regulates Lmna transcription. PMID: 23430973
46. Signaling defects that impair autophagy underlie pathogenesis of dilated cardiomyopathy arising from LMNA mutation PMID: 23044536
47. Cardiac and skeletal muscle pathology in lamin A-deficient mice can be attributed to elevated MTORC1 signaling leading to impairment of autophagic flux. PMID: 23064282
48. the physiological function of lamin A proteins is important for events that occur in the compartment of promyelocytic leukaemia bodies PMID: 22443097
49. Study identified LBR- and lamin-A/C-dependent mechanisms tethering heterochromatin to the nuclear envelope. The two tethers are sequentially used during cellular differentiation and development: first the LBR- and then the lamin-A/C-dependent tether. PMID: 23374351
50. LMNA missense mutation caused abnormal p38alpha signaling in dilated cardiomyopathy. PMID: 22773734

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KEGG: mmu:16905

STRING: 10090.ENSMUSP00000029699

UniGene: Mm.243014