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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 functional integrity of lamin and nesprin-1 is thus required to modulate the FHOD1 (show FHOD1 Proteins) 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.
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 (show Zmpste24 Proteins) cells does not lie in the repair of DNA damage per se.
pathogenic gene mutations in LMNA and MYBPC3 (show MYBPC3 Proteins) 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 (show ITK Proteins) and MET in prostate cancer tissue, since a decrease in lamin A/C expression in Gleason score (GS) 4 (show SPDYA Proteins) is likely associated with the EMT (show ITK Proteins) 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 (show ACAN Proteins) in the UK population, indicating MDC1A as the most common CMD (show ACAN Proteins) subtype (37.35%).
THE ROLE OF LMNA MUTATIONS IN MYOGENIC DIFFERENTIATION OF PRIMARY SATELLITE CELLS AND C2C12 CELLS.
Itm2a (show ITM2A Proteins) 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.
our findings indicate that altered mTOR (show FRAP1 Proteins) 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 (show FRAP1 Proteins) 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 (show SMAD1 Proteins) signaling, leading to altered activation of CTGF/CCN2 (show CTGF Proteins) 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 (show SUMO1 Proteins) conjugation of RB and Lamin A/C is modulated by the SUMO protease SENP1 (show SENP1 Proteins) and that sumoylation of both proteins is required for their interaction.
findings reveal coordination of AGO2 (show EIF2C2 Proteins) and LaminB function to dictate genome architecture and thereby regulate gene expression
The loss of lamin-B (show LMNB1 Proteins) 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.
prometaphase spindle can be made robust to natural fluctuations in motor ratios by a disassembling lamin-B (show LMNB1 Proteins) envelope.
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.
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 (show PTH Proteins)-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 (show LMNB2 Proteins) 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
Both anti-lamin A/C and anti-lamin B (show LMNB1 Proteins) 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