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Nuclear lamin

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https://www.readbyqxmd.com/read/27911330/fhl1b-interacts-with-lamin-a-c-and%C3%A2-emerin-at-the-nuclear-lamina-and%C3%A2-is%C3%A2-misregulated-in-emery-dreifuss-muscular-dystrophy
#1
Esma Ziat, Kamel Mamchaoui, Maud Beuvin, Isabelle Nelson, Feriel Azibani, Simone Spuler, Gisèle Bonne, Anne T Bertrand
BACKGROUND: Emery-Dreifuss muscular dystrophy (EDMD) is associated with mutations in EMD and LMNA genes, encoding for the nuclear envelope proteins emerin and lamin A/C, indicating that EDMD is a nuclear envelope disease. We recently reported mutations in FHL1 gene in X-linked EDMD. FHL1 encodes FHL1A, and the two minor isoforms FHL1B and FHL1C. So far, none have been described at the nuclear envelope. OBJECTIVE: To gain insight into the pathophysiology of EDMD, we focused our attention on the poorly characterized FHL1B isoform...
November 29, 2016: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/27901466/differentiation-alters-stem-cell-nuclear-architecture-mechanics-and-mechano-sensitivity
#2
Su-Jin Heo, Tristan P Driscoll, Stephen D Thorpe, Nandan L Nerurkar, Brendon M Baker, Michael T Yang, Christopher S Chen, David A Lee, Robert L Mauck
Mesenchymal stem cell (MSC) differentiation is mediated by soluble and physical cues. In this study, we investigated differentiation induced transformations in MSC cellular and nuclear biophysical properties and queried their role in mechanosensation. Our data show that nuclei in differentiated bovine and human MSCs stiffen and become resistant to deformation. This attenuated nuclear deformation was governed by restructuring of Lamin A/C and increased heterochromatin content. This change in nuclear stiffness sensitized MSCs to mechanical loading induced calcium signaling and differentiated marker expression...
November 30, 2016: ELife
https://www.readbyqxmd.com/read/27883036/the-mevalonate-pathway-regulates-primitive-streak-formation-via-protein-farnesylation
#3
Yoshimi Okamoto-Uchida, Ruoxing Yu, Norio Miyamura, Norie Arima, Mari Ishigami-Yuasa, Hiroyuki Kagechika, Suguru Yoshida, Takamitsu Hosoya, Makiko Nawa, Takeshi Kasama, Yoichi Asaoka, Reiner Wimmer Alois, Ulrich Elling, Josef M Penninger, Sachiko Nishina, Noriyuki Azuma, Hiroshi Nishina
The primitive streak in peri-implantation embryos forms the mesoderm and endoderm and controls cell differentiation. The metabolic cues regulating primitive streak formation remain largely unknown. Here we utilised a mouse embryonic stem (ES) cell differentiation system and a library of well-characterised drugs to identify these metabolic factors. We found that statins, which inhibit the mevalonate metabolic pathway, suppressed primitive streak formation in vitro and in vivo. Using metabolomics and pharmacologic approaches we identified the downstream signalling pathway of mevalonate and revealed that primitive streak formation requires protein farnesylation but not cholesterol synthesis...
November 24, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27875985/nuclear-envelope-structural-defect-underlies-the-main-cause-of-aneuploidy-in-ovarian-carcinogenesis
#4
Callinice D Capo-Chichi, Toni M Yeasky, Elizabeth R Smith, Xiang-Xi Xu
BACKGROUND: The Cancer Atlas project has shown that p53 is the only commonly (96 %) mutated gene found in high-grade serous epithelial ovarian cancer, the major histological subtype. Another general genetic change is extensive aneuploidy caused by chromosomal numerical instability, which is thought to promote malignant transformation. Conventionally, aneuploidy is thought to be the result of mitotic errors and chromosomal nondisjunction during mitosis. Previously, we found that ovarian cancer cells often lost or reduced nuclear lamina proteins lamin A/C, and suppression of lamin A/C in cultured ovarian epithelial cells leads to aneuploidy...
November 22, 2016: BMC Cell Biology
https://www.readbyqxmd.com/read/27865926/emerin-suppresses-notch-signaling-by-restricting-the-notch-intracellular-domain-to-the-nuclear-membrane
#5
Byongsun Lee, Tae-Hee Lee, Jaekyung Shim
Emerin is an inner nuclear membrane protein that is involved in maintaining the mechanical integrity of the nuclear membrane. Increasing evidence supports the involvement of emerin in the regulation of gene expression; however, its precise function remains to be elucidated. Here, we show that emerin downregulated genes downstream of Notch signaling, which are activated exclusively by the Notch intracellular domain (NICD). Deletion mutant experiments revealed that the transmembrane domain of emerin is important for the inhibition of Notch signaling...
November 16, 2016: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/27858498/sun1-splice-variants-sun1_888-sun1_785-and-predominant-sun1_916-variably-function-in-directional-cell-migration
#6
Yu Nishioka, Hiromasa Imaizumi, Junko Imada, Jun Katahira, Nariaki Matsuura, Miki Hieda
The LINC complex is a multifunctional protein complex that is involved in various processes at the nuclear envelope, such as nuclear migration, mechanotransduction and chromatin tethering in the meiotic phase. However, it remains unknown how these functions are regulated in different cell contexts. An inner nuclear membrane component of the LINC complex, SUN1, is ubiquitously expressed. The human SUN1 gene produces over 10 variants by alternative splicing. Although functions of SUN1 are relatively well characterized, functional differences among SUN1 splice variants are poorly characterized...
November 18, 2016: Nucleus
https://www.readbyqxmd.com/read/27854297/prelamin-a-accumulation-attenuates-rac1-activity-and-increases-the-intrinsic-migrational-persistence-of-aged-vascular-smooth-muscle-cells
#7
Lauren J Porter, Mark R Holt, Daniel Soong, Catherine M Shanahan, Derek T Warren
Vascular smooth muscle cell (VSMC) motility is essential during both physiological and pathological vessel remodeling. Although ageing has emerged as a major risk factor in the development of cardiovascular disease, our understanding of the impact of ageing on VSMC motility remains limited. Prelamin A accumulation is known to drive VSMC ageing and we show that presenescent VSMCs, that have accumulated prelamin A, display increased focal adhesion dynamics, augmented migrational velocity/persistence and attenuated Rac1 activity...
November 15, 2016: Cells
https://www.readbyqxmd.com/read/27854160/lamin-b1-mediated-demyelination-linking-lamins-lipids-and-leukodystrophies
#8
Quasar S Padiath
Autosomal Dominant Leukodystrophy (ADLD), a fatal adult onset demyelinating disorder, is the only human disease that has been linked to mutations of the nuclear lamina protein, lamin B1, and is primarily caused by duplications of the LMNB1 gene. Why CNS myelin is specifically targeted and the mechanisms underlying ADLD are unclear. Recent work from our group has demonstrated that over expression of lamin B1 in oligodendrocytes, the myelin producing cells in the CNS, resulted in age dependent epigenetic modifications, transcriptional down-regulation of lipogenic gene expression and significant reductions of myelin-enriched lipids...
November 17, 2016: Nucleus
https://www.readbyqxmd.com/read/27845687/extracellular-matrix-remodeling-and-transforming-growth-factor-%C3%AE-signaling-abnormalities-induced-by-lamin-a-c-variants-that-cause-lipodystrophy
#9
Caroline Le Dour, Wei Wu, Véronique Béréziat, Jacqueline Capeau, Corinne Vigouroux, Howard J Worman
Mutations in the lamin A/C gene encoding nuclear lamins A and C (lamin A/C) cause familial partial lipodystrophy type 2 (FPLD2) and related lipodystrophy syndromes. These are mainly characterized by redistribution of adipose tissue associated with insulin resistance. Several reports suggest that alterations in the extracellular matrix of adipose tissue leading to fibrosis play a role in the pathophysiology of lipodystrophy syndromes. However, the extent of extracellular matrix alterations in FPLD2 remains unknown...
November 14, 2016: Journal of Lipid Research
https://www.readbyqxmd.com/read/27835913/circulation-autoantibody-against-lamin-a-c-in-patients-with-sj%C3%A3-gren-s-syndrome
#10
Wen Zhang, Chunyan Zhang, Peng Chen, Chunhe Yang, Xianfeng Gan, Muhammad Hussain, Yiping Xun, Yaping Tian, Hongwu Du
Lamin A/C proteins are major components of nuclear laminae and were encoded by the LMNA gene. Recent studies have found that in addition to provides nuclear-membrane strength; it also regulates the gene expression. Lamin A/C has been confirmed as an autoantigen in RA, SLE and vasculitis. Anti-Lamin A/C antibodies also have been found by indirect immunofluorescence method. In this study, we used various research methods to confirm Lamin A/C is an autoantigen in Han Chinese patients with confirmed Sjögren's syndrome (SS)...
November 9, 2016: Oncotarget
https://www.readbyqxmd.com/read/27830109/pelger-hu%C3%A3-t-anomaly-and-greenberg-skeletal-dysplasia-lbr-associated-diseases-of-cholesterol-metabolism
#11
Elizabeth M Turner, Christian Schlieker
Lamin B Receptor (LBR) is an inner nuclear membrane protein associated with the rare human diseases Pelger-Huët anomaly and Greenberg skeletal dysplasia. A new study has used CRISPR/Cas9-mediated genetic manipulations in a human cell system to determine that the molecular etiology of these previously poorly understood disorders is a defect in cholesterol synthesis due to loss of LBR-associated sterol C14 reductase activity. The study furthermore determined that disease-associated LBR point mutations reduce sterol C14 reductase activity by decreasing the affinity of LBR for the reducing agent NADPH...
2016: Rare Diseases
https://www.readbyqxmd.com/read/27810932/activity-dependent-dynamics-of-the-transcription-factor-creb-in-cortical-neurons-revealed-by-single-molecule-imaging
#12
Hironobu Kitagawa, Noriyuki Sugo, Masatoshi Morimatsu, Yoshiyuki Arai, Toshio Yanagida, Nobuhiko Yamamoto
: Transcriptional regulation is crucial for neuronal activity-dependent processes that govern neuronal circuit formation and synaptic plasticity. An intriguing question is how neuronal activity influences the spatiotemporal interactions between transcription factors and their target sites. Here we investigated the activity dependence of DNA binding and dissociation events of cAMP-response element binding protein (CREB), a principal factor in activity-dependent transcription, in mouse cortical neurons using a single-molecule imaging technique...
November 3, 2016: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/27803806/recent-advances-in-understanding-the-role-of-lamins-in-health-and-disease
#13
REVIEW
Sita Reddy, Lucio Comai
Lamins are major components of the nuclear lamina, a network of proteins that supports the nuclear envelope in metazoan cells. Over the past decade, biochemical studies have provided support for the view that lamins are not passive bystanders providing mechanical stability to the nucleus but play an active role in the organization of the genome and the function of fundamental nuclear processes. It has also become apparent that lamins are critical for human health, as a large number of mutations identified in the gene that encodes for A-type lamins are associated with tissue-specific and systemic genetic diseases, including the accelerated aging disorder known as Hutchinson-Gilford progeria syndrome...
2016: F1000Research
https://www.readbyqxmd.com/read/27803112/intermediate-filaments-structure-and-assembly
#14
REVIEW
Harald Herrmann, Ueli Aebi
Proteins of the intermediate filament (IF) supergene family are ubiquitous structural components that comprise, in a cell type-specific manner, the cytoskeleton proper in animal tissues. All IF proteins show a distinctly organized, extended α-helical conformation prone to form two-stranded coiled coils, which are the basic building blocks of these highly flexible, stress-resistant cytoskeletal filaments. IF proteins are highly charged, thus representing versatile polyampholytes with multiple functions. Taking vimentin, keratins, and the nuclear lamins as our prime examples, we present an overview of their molecular and structural parameters...
November 1, 2016: Cold Spring Harbor Perspectives in Biology
https://www.readbyqxmd.com/read/27802161/elys-regulates-the-localization-of-lbr-by-modulating-its-phosphorylation-state
#15
Yasuhiro Mimura, Masatoshi Takagi, Michaela Clever, Naoko Imamoto
Lamin B receptor (LBR), an inner nuclear membrane (INM) protein, contributes to the functional integrity of the nucleus by tethering heterochromatin to the nuclear envelope. We have previously reported that the depletion of embryonic large molecule derived from yolk sac (ELYS; also known as AHCTF1), a component of the nuclear pore complex, from cells perturbs the localization of LBR to the INM, but little is known about the underlying molecular mechanism. In this study, we found that the depletion of ELYS promoted LBR phosphorylation at the residues known to be phosphorylated by cyclin-dependent kinase (CDK) and serine/arginine protein kinases 1 and 2 (SRPK1 and SRPK2, respectively)...
November 15, 2016: Journal of Cell Science
https://www.readbyqxmd.com/read/27798680/loukoumasomes-are-distinct-subcellular-structures-from-rods-and-rings-and-are-structurally-associated-with-map2-and-the-nuclear-envelope-in-retinal-cells
#16
Jake W Noble, Diana V Hunter, Calvin D Roskelley, Edward K L Chan, Julia Mills
"Rods and rings" (RR) and loukoumasomes are similarly shaped, subcellular macromolecular structures with as yet unknown function. RR, so named because of their shape, are formed in response to inhibition in the GTP or CTP synthetic pathways and are highly enriched in the two key enzymes of the nucleotide synthetic pathway. Loukoumasomes also occur as linear and toroidal bodies and were initially inferred to be the same as RR, largely due to their shared shape and size and the fact that it was unclear if they shared the same subcomponents...
2016: PloS One
https://www.readbyqxmd.com/read/27798236/lamin-b1-contributes-to-the-proper-timing-of-epicardial-cell-migration-and-function-during-embryonic-heart-development
#17
Joseph R Tran, Xiaobin Zheng, Yixian Zheng
Lamin proteins form a meshwork beneath the nuclear envelope and contribute to many different cellular processes. Mutations in lamins cause defective organogenesis in mouse models and human diseases that affect adipose, brain, skeletal muscle, and the heart. In vitro cell culture studies have shown that lamins help maintain nuclear shape and facilitate cell migration. However, whether these defects contribute to improper tissue building in vivo requires further clarification. By studying the heart epicardium during embryogenesis, we show that Lb1 null epicardial cells exhibit an in vivo and in vitro migratory delay...
October 19, 2016: Molecular Biology of the Cell
https://www.readbyqxmd.com/read/27793050/the-lamina-nf-y-protein-complex-reveals-an-unknown-transcriptional-mechanism-on-cell-proliferation
#18
Lucia Cicchillitti, Isabella Manni, Carmine Mancone, Giulia Regazzo, Manuela Spagnuolo, Tonino Alonzi, Fabrizio Carlomosti, Maria Lucia Dell'Anna, Giulia Dell'Omo, Mauro Picardo, Paolo Ciana, Maurizio C Capogrossi, Marco Tripodi, Alessandra Magenta, Maria Giulia Rizzo, Aymone Gurtner, Giulia Piaggio
Lamin A is a component of the nuclear matrix that also controls proliferation by largely unknown mechanisms. NF-Y is a ubiquitous protein involved in cell proliferation composed of three subunits (-YA -YB -YC) all required for the DNA binding and transactivation activity. To get clues on new NF-Y partner(s) we performed a mass spectrometry screening of proteins that co-precipitate with the regulatory subunit of the complex, NF-YA. By this screening we identified lamin A as a novel putative NF-Y interactor. Co-immunoprecipitation experiments and confocal analysis confirmed the interaction between the two endogenous proteins...
October 26, 2016: Oncotarget
https://www.readbyqxmd.com/read/27791462/deregulation-of-focal-adhesion-formation-and-cytoskeletal-tension-due-to-loss-of-a-type-lamins
#19
Tobias D J Corne, Tom Sieprath, Jonathan Vandenbussche, Danahe Mohammed, Mariska Te Lindert, Kris Gevaert, Sylvain Gabriele, Katarina Wolf, Winnok H De Vos
The nuclear lamina mechanically integrates the nucleus with the cytoskeleton and extracellular environment and regulates gene expression. These functions are exerted through direct and indirect interactions with the lamina's major constituent proteins, the A-type lamins, which are encoded by the LMNA gene. Using quantitative stable isotope labeling-based shotgun proteomics we have analyzed the proteome of human dermal fibroblasts in which we have depleted A-type lamins by means of a sustained siRNA-mediated LMNA knockdown...
October 28, 2016: Cell Adhesion & Migration
https://www.readbyqxmd.com/read/27783855/diseases-of-the-nucleoskeleton
#20
James M Holaska
The nucleus is separated from the cytosol by the nuclear envelope, which is a double lipid bilayer composed of the outer nuclear membrane and the inner nuclear membrane. The intermediate filament proteins lamin A, lamin B, and lamin C form a network underlying the inner nuclear membrane. This proteinaceous network provides the nucleus with its strength, rigidity, and elasticity. Positioned within the inner nuclear membrane are more than 150 inner nuclear membrane proteins, many of which interact directly with lamins and require lamins for their inner nuclear membrane localization...
September 15, 2016: Comprehensive Physiology
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