• Expressed in differentiated cells, A-type lamins originate from the LMNA gene. (wikipedia.org)
  • Thus far among the lamins, these have been described only for LMNA , which maps to chromosome 1q21.2 and encodes lamin A and lamin C through alternative splicing. (bmj.com)
  • Recently, MAD has been shown to be caused by a specific mutation (R527H) in LMNA , which encodes lamins A and C. (bmj.com)
  • Mammalian genomes contain 3 lamin genes: LMNA, which encodes lamin A and lamin C (A-type lamins), as well as LMNB1 and LMNB2 that encode lamin B1 and B2 (B-type lamins), respectively. (aad.org)
  • EDMD2/EDMD3 is due to mutations (autosomal dominant and autosomal recessive, respectively) in the LMNA gene that codes for lamins A and C. Mutations in LMNA occur throughout the gene and can cause several different phenotypes (see Causes ). (medscape.com)
  • Lamins A and C, in contrast, arise through alternative splicing of the same primary transcript encoded by the LMNA gene, expression of which is developmentally regulated. (rupress.org)
  • Mutations in the LMNA gene, encoding nuclear lamins A and C, cause a diverse range of diseases collectively called laminopathies. (jefferson.edu)
  • The overall goal of the project is to reveal pathogenic mechanisms of LMNA cardiomyopathy by focusing on two main cell types in the heart: cardiac myocytes and cardiac fibroblasts. (jefferson.edu)
  • Cell type-specific function of LMNA in the development of LMNA cardiomyopathy. (jefferson.edu)
  • A CMD Subtype that results from gene defects in the lamin A/C (LMNA) gene affectings production of lamin A and lamin C prteins within the nucleus of cells. (cmdir.org)
  • A-type lamins encoded by the LMNA gene are mainly responsible for nucleus stiffness and morphology, and their mutations are linked to several diseases. (tuni.fi)
  • The different A-type lamin isoforms result from the alternative splicing of the LMNA gene product pre-lamin A. CRISPR/Cas9 is a novel genome editing tool that can be used to generate knock-out cell mod-els to study the importance of specific genes, such as LMNA. (tuni.fi)
  • In this thesis, the aims were to establish LMNA knockout cell line and to study the importance of A-type lamins on nucleus morphology. (tuni.fi)
  • Endogenous lamin A expression was mostly unaffected by the LMNA modifi-cations. (tuni.fi)
  • and mutations in the LMNA gene encoding LAMIN TYPE A, a NUCLEAR LAMINA protein. (bvsalud.org)
  • Communication n° 568 Introduction Mutations in LMNA encoding Lamins A/C are responsible of several disorders named laminopathies involving striated muscles as well as adipose, nervous, cutaneous and skeletal tissues. (myobase.org)
  • Lamins, also known as nuclear lamins are fibrous proteins in type V intermediate filaments, providing structural function and transcriptional regulation in the cell nucleus. (wikipedia.org)
  • Nuclear lamins interact with inner nuclear membrane proteins to form the nuclear lamina on the interior of the nuclear envelope. (wikipedia.org)
  • Lamin proteins are involved in the disassembling and reforming of the nuclear envelope during mitosis, the positioning of nuclear pores, and programmed cell death. (wikipedia.org)
  • Further investigations found evidence that supports that all IF proteins arose from a common lamin-like ancestor. (wikipedia.org)
  • however, the presence of lamins is not a requirement for simultaneously containing IF proteins. (wikipedia.org)
  • Furthermore, sequence comparisons between lamins and IF proteins support that an amino-acid sequence that is characteristic of lamins is found in early forms of IF proteins. (wikipedia.org)
  • Similar to other IF proteins, lamins self-assemble into more complex structures. (wikipedia.org)
  • The nuclear lamina consists of a two-dimensional matrix of proteins located next to the inner nuclear membrane. (nih.gov)
  • The lamin family of proteins make up the matrix and are highly conserved in evolution. (nih.gov)
  • During mitosis, the lamina matrix is reversibly disassembled as the lamin proteins are phosphorylated. (nih.gov)
  • Lamin proteins are thought to be involved in nuclear stability, chromatin structure and gene expression. (nih.gov)
  • Inner nuclear membrane proteins interact with chromosomes in the nucleus and are important for chromosome activity. (nih.gov)
  • Lamin A/C proteins have key roles in nuclear structural integrity and chromosomal stability. (mdpi.com)
  • There are a number of inner nuclear envelope proteins that may have roles in chromosome location and anchorage, e.g. emerin and A-type lamins. (portlandpress.com)
  • Lamins are intermediate filament proteins comprising a major structural component of the nuclear lamina, which underlies the inner membrane of the nuclear envelope in most somatic cells. (bmj.com)
  • Disorders caused by defects in the nuclear lamina associated proteins are referred to as the laminopathies. (bmj.com)
  • The nuclear lamina (NL) is present in all animals and is composed of type V intermediate filaments called lamins plus lamin associated proteins. (aad.org)
  • Spatial changes in nuclear lamina coincide with redistribution of lamin A proteins and local reduction in protein mobility in senescent cell. (fightaging.org)
  • This complex includes nuclear membrane integral and associated proteins including emerin, lamin A/C, SUN1, SUN2, nesprin-1, and nesprin-2 that are proposed to form a mechanical link between the nucleoskeleton and cytoskeleton. (medscape.com)
  • Emerin is a ubiquitous inner nuclear membraneprotein, presentin nearly all cell types, although its highest expression is in skeletal and cardiacmuscle.Emerin binds to many nuclear proteins, including several gene-regulatory proteins (eg, barrier-to-autointegration factor, germ cell-less, Btf), nesprins (proteins that act as molecular scaffolds), F-actin, and lamins. (medscape.com)
  • Whatever the true mechanism, the discovery of mutations in several different nuclear membrane proteins that cause similar diseases will likely eventually lead to a better understanding of nuclear membrane physiology and the pathophysiology of diseases caused by mutations in these proteins. (medscape.com)
  • Lamins are type V intermediate fila-ment proteins that form the nuclear lamina and establish the physical connection between the nucleus and the cytoskeleton. (tuni.fi)
  • It contains lamins and lamin-associated proteins, including many integral proteins of the INM, chromatin modifying proteins, transcriptional repressors and structural proteins. (huji.ac.il)
  • A fraction of lamins is also present in the nucleoplasm, where it forms stable complexes and is associated with specific nucleoplasmic proteins. (huji.ac.il)
  • The lamins and their associated proteins are required for most nuclear activities, mitosis and for linking the nucleoplasm to all major cytoskeletal networks in the cytoplasm. (huji.ac.il)
  • Caspases are essential in cells for apoptosis , one of the main types of programmed cell death in development and most other stages of adult life, and have been termed "executioner" proteins for their roles in the cell. (wikidoc.org)
  • It is composed of three different types of proteins which are organized and regulated in time and space. (microbiologynotes.org)
  • Actually, in budding fungus, telomeres are destined to the nuclear Delphinidin chloride envelope reversibly, and little ubiquitin-like modifier proteins (SUMO)-reliant association using the nuclear periphery was suggested to restrain destined telomerase (13). (lavoixdesrroms.org)
  • Mutations in lamin genes can result in several genetic laminopathies, which may be life-threatening. (wikipedia.org)
  • Studies of lamins became more popular in the 1990s when it was discovered that mutations in the genes that code for lamins can be related to muscular dystrophies, cardiomyopathies, and neuropathies. (wikipedia.org)
  • Vertebrate lamins consist of two types, A and B. Mutations in this gene are associated with acquired partial lipodystrophy. (nih.gov)
  • A combination of mutations in Lem2 and Bqt4, which encodes a nuclear membrane protein that anchors telomeres to the nuclear membrane, caused synthetic lethality. (nih.gov)
  • In the last decade, a number of diseases associated with tissue degeneration and premature aging have been linked with mutations in lamin A or emerin. (portlandpress.com)
  • Despite highly aberrant nuclear distributions of A-type lamins and emerin in lymphoblastoid cell lines derived from patients with emerin or lamin A mutations, little or no change in chromosome location was detected. (portlandpress.com)
  • A disparate group of seemingly unrelated diseases with different affected organ systems has been attributed to lamin A/C mutations. (bmj.com)
  • Novelli et al analysed lamin A/C for mutations in five consanguineous Italian families with MAD. (bmj.com)
  • Changes in the shape of the nuclear lamina are exhibited in senescent cells, as well as in cells expressing mutations in lamina genes. (fightaging.org)
  • Moreover, with this method mutations in lamina genes were significantly distinct from cells with wild-type genes. (fightaging.org)
  • EDMD1 is caused by mutations in the EMD gene on the X chromosome that codes for the nuclear envelope protein emerin. (medscape.com)
  • New mutations have been found in the synaptic nuclear envelope protein 1 ( SYNE1 ) gene and in the synaptic nuclear envelope protein 2 ( SYNE2 ) gene in a few families, also termed Nesprin-1 and Nesprin-2, respectively. (medscape.com)
  • The mutations being studied affect a class of molecules called A-type nuclear lamins, and the lab is exploring their roles in health and disease. (lifeboat.com)
  • This review concentrates mainly on lamins, their structure and their roles in DNA replication, chromatin organization, adult stem cell differentiation, aging, tumorogenesis and the lamin mutations leading to laminopathic diseases. (huji.ac.il)
  • Mutations in the gene encoding lamin A/C cause autosomal dominant Emery-Dreifuss muscular dystrophy. (medscape.com)
  • LBR gene mutations involved in Greenberg dysplasia lead to loss of the sterol reductase function of the lamin B receptor, and research suggests that this loss causes the condition. (medlineplus.gov)
  • It wasn't until 1986 that an analysis of lamin cDNA clones across a variety of species supported that lamins belong to the intermediate filament (IF) protein family. (wikipedia.org)
  • Lamin B2 contributes to the proliferation of bladder cancer cells via activating the expression of cell division cycle‑associated protein 3. (nih.gov)
  • Lem2 and Man1 are conserved members of the LEM-domain nuclear membrane protein family. (nih.gov)
  • These genetic interactions imply that Lem2 cooperates with the nuclear membrane protein network to regulate genome stability. (nih.gov)
  • Lamin A/C cumulative protein expression of all variants is reported by semi-quantitative Western blotting. (mdpi.com)
  • Western blot analysis on extracts of both embryonic fibroblasts and liver nuclear membranes from homozygous mice confirmed that no detectable protein was expressed from this allele. (jax.org)
  • The mutation activates an alternative splice site, resulting in accumulation of an abnormal lamin A-derived protein named progerin. (aad.org)
  • Progerin lacks this crucial cleavage site, resulting in a permanently farnesylated protein that accumulates in, and causing thickening of, the nuclear membrane. (aad.org)
  • The nuclear lamina is a thin (20 nm) yet insoluble protein meshwork that, in higher cells, lines the nucleoplasmic face of the nuclear envelope (NE). (rupress.org)
  • have previously shown that reassembly of B-type lamins is under the control of both protein phosphatase 1 (PP1) and an A-kinase anchoring protein, AKAP149. (rupress.org)
  • The latter is a membrane protein localized to both the ER and nuclear membranes, and contains a specific binding site for PP1. (rupress.org)
  • Here, we discover a previously unknown type of SBD in four independent families caused by bi-allelic loss-of-function pathogenic variants in TMEM53 , which encodes a nuclear envelope transmembrane protein. (nature.com)
  • In this study, we discover a previously unknown type of SBD and identify its causal gene, TMEM53 , which encodes nuclear envelope transmembrane (NET) protein 53 (TMEM53, also known as NET4). (nature.com)
  • In addition, N-terminal deletion mutation of 20 amino acids or fluoro-phore tags in the lamin A protein delayed cell proliferation but didn't remarkably affect the nu-cleus morphology. (tuni.fi)
  • Multiple and surprising new functions for emerin, a nuclear membrane protein. (medscape.com)
  • These different types of cytoskeletal fibers are polymers which are made up of small protein subunits and held together by non-covalent interactions. (microbiologynotes.org)
  • Lamin protein. (microbiologynotes.org)
  • The nuclear membranes surrounding fish and frog oocyte germinal vesicles (GVs) are supported by the lamina, an internal, mesh-like structure that consists of the protein lamin B3. (elsevierpure.com)
  • Two-dimensional (2-D) electrophoresis of GV protein revealed two major spots of lamin B3 with different isoelectric points (5.9 and 6.1). (elsevierpure.com)
  • The former spot disappeared when the native lamina was incubated with lambda phage protein phosphatase (λ-PP), indicating that a portion of the lamin protein was already phosphorylated in the goldfish GV-stage oocytes. (elsevierpure.com)
  • Lamins are fundamental structural the different parts of the nuclear lamina, an intermediate filament meshwork that is situated under the internal nuclear membrane, attaching chromatin domains towards the nuclear periphery and localizing some nuclear envelope Delphinidin chloride protein. (lavoixdesrroms.org)
  • Presence of a protein immunologically related to lamin B in the postsynaptic membrane of Torpedo marmorata electrocyte. (silverchair.com)
  • This protein which thus shares epitopes with lamin B has a relative molecular mass of 54 kD, an acidic IP of 5.4. (silverchair.com)
  • This gene provides instructions for making a protein called the lamin B receptor. (medlineplus.gov)
  • During cholesterol synthesis, the sterol reductase function of the lamin B receptor allows the protein to perform one of several steps that convert a molecule called lanosterol to cholesterol. (medlineplus.gov)
  • Inner nuclear membrane protein LBR preferentially interacts with DNA secondary structures and nucleosomal linker. (medlineplus.gov)
  • During this time period, investigations of rat liver nuclei revealed that lamins have an architectural relationship with chromatin and nuclear pores. (wikipedia.org)
  • A-type lamins also associate with chromatin in the nuclear interior, away from the peripheral nuclear lamina. (frontiersin.org)
  • The hot-spot lamin A R482W mutation causing familial partial lipodystrophy of Dunnigan-type (FPLD2), affects lamin A association with chromatin at the nuclear periphery and in the nuclear interior, and is associated with 3-dimensional (3D) rearrangements of chromatin. (frontiersin.org)
  • Association of A-type lamins with chromatin at the nuclear periphery and in the nuclear interior. (frontiersin.org)
  • (D) Nucleoplasmic lamin A interacts with chromatin in the nuclear interior. (frontiersin.org)
  • E , Left) Computational 3D model of the genome in a diploid human fibroblast nucleus taking into account genome-wide chromosomal interactions and interactions between chromatin and the nuclear periphery. (frontiersin.org)
  • Although their nuclear functions are currently being elucidated, it has been hypothesised that they are involved in membrane support, pore arrangement, envelope assembly, and chromatin organisation. (bmj.com)
  • The first suggests that disruption of the inner nuclear membrane and the nuclear lamina causes disorganization of nuclear chromatin and gene expression, while the second proposes that the mechanical strength of the cell nucleus is disrupted when the nuclear lamina is weakened leading to structural and signaling defects in mechanically stressed tissue such as muscle and heart. (medscape.com)
  • The lamina is intimately associated with both the inner nuclear membrane and underlying chromatin, while at the same time providing anchoring sites for nuclear pore complexes ( Gerace and Burke 1988 ). (rupress.org)
  • During mitosis, PP1 appears largely chromatin bound, but in telophase it is recruited to the nuclear periphery to dephosphorylate B-type lamins and thereby enable polymerization and lamina assembly. (rupress.org)
  • The bands are characterized by low gene density, contain predominantly tissue-specific genes, and are represented by silent chromatin types in various tissues. (sdbonline.org)
  • Genomic regions associating with other nuclear compartments may similarly define distinct classes of chromatin domains. (4dnucleome.org)
  • c) Micronuclei type after mitosis because of impaired segregation of DNA during mitosis, from chromatin bridges and lagging chromosomes/chromatin fragments (further explanation, Supplementary Text message). (biopaqc.com)
  • There, A- and B-type lamins interact with chromatin via lamina-associated domains (LADs). (collaslab.org)
  • We investigate the mechanisms of lamin interactions with chromatin during adipogenic differentiation, the relationship between LADs and TADs as genome organizers, the origin and purpose of LAD heterogeneity, and how LADs contribute to the definition of adipose cell identity. (collaslab.org)
  • The mechanisms by which lamin B3 is transported into GVs and is assembled to form the nuclear lamina are not well understood. (elsevierpure.com)
  • The results of these experiments indicated that the N-terminal domain plays important roles in both nuclear transport and assembly of lamin B3 to form the nuclear lamina. (elsevierpure.com)
  • Lamins were first identified in the cell nucleus, using electron-microscopy. (wikipedia.org)
  • These complex structures allow nuclear lamins to perform their specialized functions in maintaining the shape of the nucleus as well as roles during mitosis and apoptosis. (wikipedia.org)
  • Chromosomal aneuploidies induced upon Lamin B2 depletion are mislocalized in the interphase nucleus. (nih.gov)
  • This nucleoplasmic lamin A environment tends to be euchromatic, suggesting distinct roles of lamin A in the regulation of gene expression in peripheral and more central regions of the nucleus. (frontiersin.org)
  • The periphery of the mammalian nucleus is delineated by the nuclear envelope and by subjacent domains of compact and repressed heterochromatin separated by more open and active regions in the vicinity of nuclear pores. (frontiersin.org)
  • NR type II is observed in multiple cells as well as nuclear tunnels transversing the nucleus. (figshare.com)
  • NOFIC: Decades of microscopy have revealed that the nucleus is not a homogeneous organelle, but rather consists of distinct compartments such as nucleoli, nuclear speckles, the nuclear lamina, among other structures. (4dnucleome.org)
  • The working hypothesis was that the depletion of A-type lamins leads to irregular nucleus shape. (tuni.fi)
  • The effect of pre-lamin A depletion on nucleus morphology was then studied by using wild type (wt) and sta-bly fluorophore-conjugated lamin-A expressing cell lines including lamin A-mutant unable to incorporate into the nuclear lamina as controls. (tuni.fi)
  • In support of the hypothesis, the results showed that the depletion of A-type lamins led to an irregular nucleus shape. (tuni.fi)
  • Conclusions: To conclude, the results support the hypothesis and demonstrate the im-portance of A-type lamins on nucleus morphology. (tuni.fi)
  • Keratin of epithelial cells, desmin and vimnectin of muscle cells, fibroblasts and WBC, neurofilament of neurons, lamin of nucleus are main intermediate filament. (microbiologynotes.org)
  • We discuss associated epigenetic and genome conformation changes elicited by the lamin A R482W mutation at the gene level. (frontiersin.org)
  • 15, 16 Recently, Novelli et al categorised mandibuloacral dysplasia as a laminopathy resulting from lamin A/C mutation. (bmj.com)
  • Mutational analysis of the lamin A/C gene revealed homozygosity for the identical R527H mutation as reported previously, but with a distinct haplotype. (bmj.com)
  • In collaboration with a group of neurologists from the Hospital de la Santa Creu i Sant Pau, Barcelona, we will analyse lamin alterations in blood plasma and lymphocytes from carriers of the mutation as well as patients at different stages of the disease. (fundacionareces.es)
  • The structure of lamins is composed of three units that are common among intermediate filaments: a central α-helical rod domain containing heptad repeats surrounded by globular N and C-terminal domains. (wikipedia.org)
  • 1][4] Most types of intermediate filaments are cytoplasmic, but one type, the lamins, are nuclear. (pearltrees.com)
  • 105:105-115) in which type III intermediate size filaments are vectorially inserted to plasma and nuclear membranes by ankyrin and lamin B, respectively. (silverchair.com)
  • This gene encodes a B type nuclear lamin. (nih.gov)
  • Through these associations, the lamins may have more expanded roles at the cellular level and control diverse functions such as DNA synthesis, gene expression, and apoptosis. (bmj.com)
  • Radial placement of beads is determined from, here, lamin A ChIP-sequencing data in fibroblasts. (frontiersin.org)
  • Progerin induced senescence, lamin B1 loss, DNA damage, and telomere shortening are prevented by TERT in primary and HGPS fibroblasts, control experiments. (elifesciences.org)
  • A ) Western blotting showing inducible expression of progerin or wild type lamin A in primary and TERT+ human fibroblasts ± doxycyclin (DOX) as indicated. (elifesciences.org)
  • B ) Immunofluorescence microscopy using V5-tag antibody (top panels) showing doxycyclin-dependent expression of v5-lamin A and v5-progerin in TERT+ human fibroblasts and localization to the nuclear periphery. (elifesciences.org)
  • Fibroblasts extracted from lamin B1 mutant mouse embryos shown early senescence (12). (lavoixdesrroms.org)
  • At the nuclear periphery, the genome is anchored to A- and B-type nuclear lamins in the form of heterochromatic lamina-associated domains. (frontiersin.org)
  • Here, we highlight features of nuclear lamin association with the genome at the nuclear periphery and in the nuclear interior. (frontiersin.org)
  • (B) Analysis of single cells however (e.g., by FISH) indicates that not all LADs mapped in cell populations are found at the nuclear periphery in all cells. (frontiersin.org)
  • Gene-poor human chromosomes are reproducibly found at the nuclear periphery in proliferating cells. (portlandpress.com)
  • A ) Immunofluorescence microscopy using v5-tag antibody showing doxycycline-dependent inducible expression of v5-progerin and its localization to the nuclear periphery. (elifesciences.org)
  • At the nuclear periphery, the nuclear lamina provides radial (center-to-periphery) functionality to the genome. (collaslab.org)
  • In mammalian somatic cells, there are four major lamins, A, B1, B2, and C. The B-type lamins are encoded by separate genes (LMNB1 and LMNB2) and, as a class, are found in the nuclei of all mammalian somatic cells. (rupress.org)
  • 4000). ( G ) Illustration showing the number of genes whose expression changed more than twofold after 28 days of lamin A or progerin expression (I, induced. (elifesciences.org)
  • No significant changes were observed upon expression of lamin A. In primary and TERT+ cells, 5 and 142 genes were differentially regulated upon progerin expression, respectively. (elifesciences.org)
  • cGAS activation generates the cyclic dinucleotide cyclic GMP-AMP (cGAMP), which in turn activates a Type I Interferon response via the adaptor Stimulator of Interferon Genes (STING)11. (biopaqc.com)
  • Current research is being performed to develop treatment methods for the aforementioned laminopathies and to investigate the role lamins play in the aging process. (wikipedia.org)
  • A lterations in the lamin (type A and B), the main fibroid component of the nuclear laminin eukaryote cells, give rise to a series of diseases known as laminopathies. (fundacionareces.es)
  • The results suggest that the successfully established pre-lamin A knock-out cell line can serve as a model of laminopathies and can be used to study the importance of A-type lamins in cellular processes. (tuni.fi)
  • A CaaX motif is found within the unique residues in prelamin A. Due to the presence of the CaaX motif, prelamin A undergoes a series of posttranslational modifications to become mature lamin A. These steps include farnesylation of the carboxyl-terminal cysteine, endoproteolytic release of the terminal amino acids, carboxymethalation of the accessible farnesylcysteine, and removal of the final fifteen residues by a zinc metalloprotease. (wikipedia.org)
  • Proteolytic cleavage by the ZMPSTE24 protease removes the last 15 amino acid residues and the hydrophobic farnesyl group to produce mature lamin A, which is found both at the NL and in the nuclear interior. (aad.org)
  • The anal-ysis of the nuclear aspect ratio showed, that the pre-lamin A -depleted nuclei were mostly cres-cent- or donut-shaped, and that their circularity, roundness and solidity differed significantly from those of the wt cells. (tuni.fi)
  • However, they were not recognized as vital components of nuclear structural support until 1975. (wikipedia.org)
  • Deliverables of this proposal include a wide range of structural and functional maps of nuclear organization, reagents for visualizing endogenous chromosome loci, a powerful pipeline for synthesis of ~100kb DNA fragments, and cell lines facilitating repeated, high-fidelity insertio of these large fragments back into selected sites in the genome. (4dnucleome.org)
  • These include Charcot-Marie-Tooth disease type 2B, 7 forms of dilated cardiomyopathy, 8 both autosomal dominant and autosomal recessive forms of Emery-Dreifuss muscular dystrophy, 9, 10 limb girdle muscular dystrophy type 1B, 11 Dunnigan-type familial partial lipodystrophy, 12- 14 and Hutchinson-Gilford progeria. (bmj.com)
  • (A) Lamina-associated domains (LADs), at the nuclear envelope, schematized from analyses of cell populations using genome-wide approaches such as DamID- or ChIP-sequencing. (frontiersin.org)
  • One major bottleneck towards a deeper understanding of nuclear organization has been the inability to convert microscopy views of nuclear compartments into genome-wide maps that show which loci are associated with which compartment, and how the chromosomal fiber traverses between compartments. (4dnucleome.org)
  • Two isoforms, lamins A and C, can be created from this gene via alternative splicing. (wikipedia.org)
  • D ) Quantification of lamin B1 levels upon progerin or lamin A expression in primary and TERT+ cells. (elifesciences.org)
  • Further studies are need-ed to elucidate the mechanisms of the phenomena as well as the effects of lamin knock-out on other cellular processes, such as mechanical force transmission and gene expression. (tuni.fi)
  • As these protofilaments aggregate, they form lamin filaments. (wikipedia.org)
  • To investigate nuclear lamin phosphorylation, we generated a monoclonal antibody (C7B8D) against Ser-28-phosphorylated GFLB3. (elsevierpure.com)
  • However, the mechanism by which nuclear translocation is regulated remains unclear. (nature.com)
  • Together, our data demonstrate that translocation of phospho-ERK to the nuclear envelope is mediated by Ser 154 -Val 155 -Ser 156 domain of cav-2 and this event is an insulin-specific action. (korea.ac.kr)
  • I'm not sure that we can expect to see the same nuclear lamellar abnormalities in stromal cells, adipocytes, vascular smooth muscle cells, or other non-stem-cell cell types, where senescence probably plays the most actively malign role in aging (as opposed to the 'negative' harm of implying stem cell attrition). (fightaging.org)
  • Loss of A-type lamin expression compromises nuclear envelope integrity leading to muscular dystrophy. (jax.org)
  • Subsequent evaluation of this family by Emery and Dreifuss in 1966 led to distinguishing this type of X-linked dystrophy from the more severe Duchenne and Becker muscular dystrophies. (medscape.com)
  • Nesprin-1 and -2 are involved in the pathogenesis of Emery Dreifuss muscular dystrophy and are critical for nuclear envelope integrity. (medscape.com)
  • Unusual type of benign x-linked muscular dystrophy. (medscape.com)
  • The Emery-Dreifuss muscular dystrophy phenotype arises from aberrant targeting and binding of emerin at the inner nuclear membrane. (medscape.com)
  • Open up in another home window Fig 1 cGAS localises to micronuclei caused by endogenous and exogenous DNA harm(a) Micronuclei type often in MEFs, connected with genome instability. (biopaqc.com)
  • Lamin B1 stain of S1 acinus illuminates multiple nuclear tunnels and the NR. Confocal stack of staining for lamin B1 (green) and DNA (DAPI, blue) in growth arrested S1 acini. (figshare.com)
  • Consultant picture with Lamin B1 (reddish colored) staining the nuclear envelope and DAPI staining DNA (blue). (biopaqc.com)
  • We identified that the Ser 154 -Val 155 -Ser 156 domain on the C-terminal of cav-2 is essential for insulin-induced phosphorylation and nuclear targeting of ERK and cav-2. (korea.ac.kr)
  • Thus, lamin phosphorylation appears to occur prior to oocyte maturation in vivo in both these species. (elsevierpure.com)
  • Taken together, our results suggest that the balance between phosphorylation by interphase lamin kinases and dephosphorylation by phosphatases regulates the conformational changes in the lamin B3 N-terminal head domain that in turn regulates the continual in vivo rearrangement and remodeling of the oocyte lamina. (elsevierpure.com)
  • Furthermore, lamin C contains six unique amino-acid residues while prelamin A contains ninety-eight residues not found in the other isoform. (wikipedia.org)
  • Lamin B2 promotes the malignant phenotype of non-small cell lung cancer cells by upregulating dimethylation of histone 3 lysine 9. (nih.gov)
  • Centromeric heterochromatin formation was augmented upon transfer to the rich medium in wild-type cells. (nih.gov)
  • A quantitative distinction of the nuclear lamina shape in cell populations was found between fresh and senescent cells, and between primary myoblasts from young and old donors. (fightaging.org)
  • The mesohyl includes a noncellular colloidal mesoglea with embedded collagen fibers, spicules and various cells, being as such a type of mesenchyme. (pearltrees.com)
  • Murine Lewis lung carcinoma (LLC) cells shaped smaller sized tumors in mice missing SK2 than in wild-type mice, and knocking down SK2 in LLC cells before implantation into mice suppressed their development. (lavoixdesrroms.org)
  • Additionally, alterations in the nuclear laminin Parkinson's disease suggest that they participate in neurodegenerative processes. (fundacionareces.es)
  • Unlike lamin C, Lamin A is generated in a precursor form called prelamin A. Prelamin A and lamin C differ in structure only at the carboxyl-terminus. (wikipedia.org)
  • The lamin A precursor also contains a CaaX motif and is initially farnesylated. (aad.org)
  • The role of lamin B2 in human diseases. (nih.gov)
  • In conclusion, the characteristic pattern of polytene chromosomes reflects partitioning of the Drosophila genome into two global types of domains with contrasting properties. (sdbonline.org)
  • It can be speculated if the depletion of A-type lamins affects the nuclear mechanotransduction. (tuni.fi)
  • Progerin migrates between lamin A and C as indicated (red arrowhead). (elifesciences.org)