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Human Lamin A/C Protein expressed in Wheat germ - ABIN1309507
Chen, Lin, Chow, Lee: Proteomic identification of Hsp70 as a new Plk1 substrate in arsenic trioxide-induced mitotically arrested cells. in Proteomics 2011
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The finding of a new LMNA pathologic gene variant in this patient increases the options for genetic testing of individuals with restrictive cardiomyopathy.
Lamin A-C interaction with Nestin and its role in the tumor senescence.Nestin stabilizes lamin A-C to protect tumor cells from senescence.
Among the 120 dilated cardiomyopathy patients 13 (10.8%) had LMNA variants. A novel recurrent LMNA E115M variant was the most frequent in familial DCM.
lamin A/C interacts with Notch signaling, thereby influencing cellular differentiation, and point mutation in LMNA could halt this interaction
Mutations in LMNA cause autosomal dominant severe heart disease, accounting for 10% of Dilated Cardiomyopathy .
ZMPSTE24-dependent cleavage of prelamin A and the eight known disease-associated ZMPSTE24 missense mutations, were examined.
The LMNA-NTRK1 fusion was likely the molecular driver of tumorigenesis and metastasis in this patient, and the observed effectiveness of crizotinib treatment provides clinical validation of this molecular target.
Three heterozygous missense mutations were identified in unrelated patients - p. W520R (c.1558T > C), p.T528R (small es, Cyrillic.1583capital ES, Cyrillic > G) and p.R190P (c.569G > C). We consider these variants as pathogenic, leading to isolated DCM with conduction defects or syndromic DCM forms with limb-girdle muscular dystrophy and Emery- Dreifuss muscular dystrophy.
The functional integrity of lamin and nesprin-1 is thus required to modulate the FHOD1 activity and the inside-out mechanical coupling that tunes the cell internal stiffness to match that of its soft, physiological-like environment.
The role of 1B and 2B domains in modulating elastic properties of lamin A.
progerin is upregulated in human dilated cardiomyopathy hearts and strongly correlates with left ventricular remodeling
Data indicate that patients with truncation mutations in LMNA (lamin A/C) had an earlier occurrence of cardiac conduction disturbance and low left ventricular ejection fraction, than those with missense mutations.
A novel truncating LMNA mutation associated with Cardiac conduction disorders and dilated cardiomyopathy was discovered in this family characterized by gender differences in clinical severity in LMNA carriers
We find no evidence for an elevated mutation rate in progerin-expressing cells. We conclude that the cellular defect in HGPS cells does not lie in the repair of DNA damage per se.
pathogenic gene mutations in LMNA and MYBPC3 alter RNA splicing and may have a role in heart disease
Patients with the heterozygous LMNA p.T10I mutation have distinct clinical features and significantly worse metabolic complications compared with other patients with atypical progeroid syndrome as well as patients with Hutchinson-Gilford progeria syndrome.
Results suggest that lamin A/C might constitute a type of epithelial marker that better signifies EMT and MET in prostate cancer tissue, since a decrease in lamin A/C expression in Gleason score (GS) 4 is likely associated with the EMT process, while the re-expression of lamin A/C in GS 5 is likely linked with MET.
Using cardiomyocytes derived from human induced pluripotent stem cells carrying different LMNA mutations as model for dilated cardiomyopathy, demonstrate that PTC124 induces translational read-through over the premature stop codon and restores production of the full-length protein.
This study represents a comprehensive report on relative frequency of CMD in the UK population, indicating MDC1A as the most common CMD subtype (37.35%).
in differentiating myoblasts, nuclear HSPB2 compartments sequester lamin A.
Data show that lamin A/C expressing cells can form an actin cap to resist nuclear deformation in response to physiological mechanical stresses.
THE ROLE OF LMNA MUTATIONS IN MYOGENIC DIFFERENTIATION OF PRIMARY SATELLITE CELLS AND C2C12 CELLS.
Itm2a knockdown is sufficient to rescue the inhibitory effects of lamin A WT and R482W mutant overexpression on 3T3-L1 differentiation.
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.
Lmna gene product might play a crucial role in the ChAT-dependent molecular differentiation cascade.
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.
Alterations in ventricular myocytes ion channel properties may contribute to arrhythmia and sudden cardiac death in LmnaN195K mutation-related Familial Dilated Cardiomyopathy.
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
results suggest that lamin A plays important roles in maintaining the osteoblast differentiation and function
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.
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
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.
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.
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.
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.
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.
administration of the exon 11 ASO reduced lamin A expression in wild-type mice and progerin expression in an HGPS mouse model.
stabilization of perinuclear actin strengthens the transient interactions of lamin A with chromatin
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
Annexin, lamin, and vimentin were identified as universal dystrophic markers
findings reveal coordination of AGO2 and LaminB function to dictate genome architecture and thereby regulate gene expression
The loss of lamin-B leads to systemic inflammation and aging.
nuclear Wash interacts with B-type Lamin (Lamin Dm0), and, like Lamin, Wash associates with constitutive heterochromatin
of single phosphorylation sites of Drosophila melanogaster lamin Dm and lamin C
Rab5 associated in vivo with nuclear Lamin and mushroom body defect (Mud), the Drosophila counterpart of nuclear mitotic apparatus protein (NuMA).
Kugelkern and lamin Dm0 affect nuclear shape without requiring filament formation or the presence of a classical nuclear lamina.
Lamin mutation is associated with neuromuscular defects and premature aging.
analysis of Drosophila and human A-type lamins
prometaphase spindle can be made robust to natural fluctuations in motor ratios by a disassembling lamin-B envelope.
Null mutants of Drosophila B-type lamin Dm(0) show aberrant tissue differentiation rather than obvious nuclear shape distortion or specific defects during cell proliferation.
Molecular interactions with lamin Dm0 may link JIL-1 kinase to nuclear morphology and integrity of nurse cells during oogenesis.
The lamin Dm0 allele Ari3 acts as an enhancer of position effect variegation of the wm4 allele in Drosophila.
Both lamin Dm0 and C. elegans Ce-lamin bind directly to histone H2A in vitro and this binding requires the nuclear localization signal
Induced expression of the farnesylated lamina proteins Lamin B and Kugelkern cause aberrant nuclear shapes and reduce the lifespan of adult flies.
developmental disorders caused by lesions in the B-type lamins and interacting proteins
results have important implications for understanding the tissue-specific regulation and functions of the lamin A gene
The induction of embryonic senescence and laminopathies in zebrafish harboring disturbed expressions of the lamin A gene, is described.
These results indicate that thyroid hormone-regulated expression of nuclear lamin A and LIII closely correlates with dedifferentiation of the epithelial cells in the X. laevis intestine.
LIII filaments appear identical to the endogenous lamina, whereas lamin B2 assembles into filaments that are organized less precisely; Lamin A induces sheets of thicker filaments on the endogenous lamina and increases the rigidity of the nuclear envelope
Ectopic expression of prelamin A in early Xenopus embryos induces apoptosis.
Both anti-lamin A/C and anti-lamin B staining were clearly present in all embryonic stages.
A study mapping the location of procine lamin type A to chromosome 4q is presented.
The nuclear lamina consists of a two-dimensional matrix of proteins located next to the inner nuclear membrane. The lamin family of proteins make up the matrix and are highly conserved in evolution. During mitosis, the lamina matrix is reversibly disassembled as the lamin proteins are phosphorylated. Lamin proteins are thought to be involved in nuclear stability, chromatin structure and gene expression. Vertebrate lamins consist of two types, A and B. Alternative splicing results in multiple transcript variants. Mutations in this gene lead to several diseases: Emery-Dreifuss muscular dystrophy, familial partial lipodystrophy, limb girdle muscular dystrophy, dilated cardiomyopathy, Charcot-Marie-Tooth disease, and Hutchinson-Gilford progeria syndrome.
70 kDa lamin
, lamin A/C-like 1
, renal carcinoma antigen NY-REN-32
, lamin C
, B-type lamin
, intermediate filament-like lamina
, lamin A
, lamin B
, lamin D
, lamin D[]
, lamin D[[m]]0
, lamin D[[o]]
, lamin Dm0
, lamin Dm[]
, lamin Dmo
, nuclear lamin
, lamin C2
, lamin A/C