scholarly journals Gene regulation networks in muscle differentiation in lamin A associated muscular dystrophy

2020 ◽  
Vol 34 (S1) ◽  
pp. 1-1
Author(s):  
Subarna Dutta
Keyword(s):  
Gene Regulation ◽  
Muscular Dystrophy ◽  
Muscle Differentiation ◽  
Lamin A ◽  
Gene Regulation Networks

scholarly journals PCAF Involvement in Lamin A/C-HDAC2 Interplay during the Early Phase of Muscle Differentiation

Cells ◽  
2020 ◽  
Vol 9 (7) ◽  
pp. 1735
Author(s):  
Spartaco Santi ◽  
Vittoria Cenni ◽  
Cristina Capanni ◽  
Giovanna Lattanzi ◽  
Elisabetta Mattioli
Keyword(s):  
Muscular Dystrophy ◽  
Early Phase ◽  
Myogenic Differentiation ◽  
Nuclear Lamina ◽  
Muscle Differentiation ◽  
Lamin A ◽  
Histone Deacetylase 2 ◽  
Muscle Gene Expression ◽  
Muscle Gene ◽  
Human Muscle Cells

Lamin A/C has been implicated in the epigenetic regulation of muscle gene expression through dynamic interaction with chromatin domains and epigenetic enzymes. We previously showed that lamin A/C interacts with histone deacetylase 2 (HDAC2). In this study, we deepened the relevance and regulation of lamin A/C-HDAC2 interaction in human muscle cells. We present evidence that HDAC2 binding to lamin A/C is related to HDAC2 acetylation on lysine 75 and expression of p300-CBP associated factor (PCAF), an acetyltransferase known to acetylate HDAC2. Our findings show that lamin A and farnesylated prelamin A promote PCAF recruitment to the nuclear lamina and lamin A/C binding in human myoblasts committed to myogenic differentiation, while protein interaction is decreased in differentiating myotubes. Interestingly, PCAF translocation to the nuclear envelope, as well as lamin A/C-PCAF interaction, are reduced by transient expression of lamin A mutated forms causing Emery Dreifuss muscular dystrophy. Consistent with this observation, lamin A/C interaction with both PCAF and HDAC2 is significantly reduced in Emery–Dreifuss muscular dystrophy myoblasts. Overall, these results support the view that, by recruiting PCAF and HDAC2 in a molecular platform, lamin A/C might contribute to regulate their epigenetic activity required in the early phase of muscle differentiation.

Gene Regulation Networks in Early Phase of Duchenne Muscular Dystrophy

2013 ◽  
Vol 10 (2) ◽  
pp. 393-400 ◽  
Author(s):  
Camilla Bernardini ◽  
Federica Censi ◽  
Wanda Lattanzi ◽  
Giovanni Calcagnini ◽  
Alessandro Giuliani
Keyword(s):  
Gene Regulation ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Early Phase ◽  
Gene Regulation Networks

scholarly journals Emerin Represses STAT3 Signaling through Nuclear Membrane-Based Spatial Control

2021 ◽  
Vol 22 (13) ◽  
pp. 6669
Author(s):  
Byongsun Lee ◽  
Seungjae Lee ◽  
Younggwang Lee ◽  
Yongjin Park ◽  
Jaekyung Shim
Keyword(s):  
Muscular Dystrophy ◽  
Nuclear Membrane ◽  
Target Genes ◽  
Janus Kinase ◽  
C2c12 Cells ◽  
Specific Gene ◽  
Lamin A ◽  
Stat3 Signaling ◽  
Exact Function ◽  
Muscle Homeostasis

Emerin is the inner nuclear membrane protein involved in maintaining the mechanical integrity of the nuclear membrane. Mutations in EMD encoding emerin cause Emery-Dreifuss muscular dystrophy (EDMD). There has been accumulating evidence that emerin regulation of specific gene expression is associated with this disease, but the exact function of emerin has still less revealing. Here, we have shown that emerin downregulates signal transducers and activators of transcription 3 (STAT3) signaling, activated exclusively by Janus-kinase (JAK). Deletion mutation experiments showed that the lamin-binding domain of emerin is essential for the inhibition of STAT3 signaling. Emerin interacted directly and co-localized with STAT3 in the nuclear membrane. Emerin knockdown induced STAT3 target genes Bcl2 and Survivin to increase cell survival signals and suppress hydrogen peroxide-induced cell death in HeLa cells. Specifically, downregulation of BAF or lamin A/C increases STAT3 signaling, suggesting that correct-localized emerin by assembling with BAF and lamin A/C acts as an intrinsic inhibitor against STAT3 signaling. In C2C12 cells, emerin knockdown induced STAT3 target gene, Pax7, and activated abnormal myoblast proliferation associated with muscle wasting in skeletal muscle homeostasis. Our results indicate that emerin downregulates STAT3 signaling by inducing retention of STAT3 and delaying STAT3 signaling in the nuclear membrane. This mechanism provides clues to the etiology of emerin-related muscular dystrophy and could be a new therapeutic target for treatment.

scholarly journals Identification of MEF2-regulated genes during muscle differentiation

2004 ◽  
Vol 20 (1) ◽  
pp. 143-151 ◽  
Author(s):  
James Paris ◽  
Carl Virtanen ◽  
Zhibin Lu ◽  
Mark Takahashi
Keyword(s):  
Gene Regulation ◽  
Transcription Factor ◽  
Transcription Factors ◽  
Neuromuscular Junctions ◽  
Muscle Differentiation ◽  
Signaling Cascades ◽  
Specific Gene ◽  
Genetic Mechanisms ◽  
Selection Parameters ◽  
First Time

Although a great deal has been elucidated concerning the mechanisms regulating muscle differentiation, little is known about transcription factor-specific gene regulation. Our understanding of the genetic mechanisms regulating cell differentiation is quite limited. Much of what has been defined centers on regulatory signaling cascades and transcription factors. Surprisingly few studies have investigated the association of genes with specific transcription factors. To address these issues, we have utilized a method coupling chromatin immunoprecipitation and CpG microarrays to characterize the genes associated with MEF2 in differentiating C2C12 cells. Results demonstrated a defined binding pattern over the course of differentiation. Filtered data demonstrated 9 clones to be elevated at 0 h, 792 at 6 h, 163 by 1 day, and 316 at 3 days. Using unbiased selection parameters, we selected a subset of 291 prospective candidates. Clones were sequenced and filtered for removal of redundancy between clones and for the presence of repetitive elements. We were able to place 50 of these on the mouse genome, and 20 were found to be located near well-annotated genes. From this list, previously undefined associations with MEF2 were discovered. Many of these genes represent proteins involved in neurogenesis, neuromuscular junctions, signaling and metabolism. The remaining clones include many full-length cDNA and represent novel gene targets. The results of this study provides for the first time, a unique look at gene regulation at the level of transcription factor binding in differentiating muscle.

scholarly journals Dysfunctional polycomb transcriptional repression contributes to lamin A/C–dependent muscular dystrophy

2020 ◽  
Vol 130 (5) ◽  
pp. 2408-2421 ◽  
Author(s):  
Andrea Bianchi ◽  
Chiara Mozzetta ◽  
Gloria Pegoli ◽  
Federica Lucini ◽  
Sara Valsoni ◽  
...  
Keyword(s):  
Muscular Dystrophy ◽  
Transcriptional Repression ◽  
Lamin A

ELECTRONIC DESIGN OF SYNTHETIC GENETIC NETWORKS

2007 ◽  
Vol 17 (10) ◽  
pp. 3507-3511 ◽  
Author(s):  
JAVIER M. BULDÚ ◽  
JORDI GARCÍA-OJALVO ◽  
ALEXANDRE WAGEMAKERS ◽  
MIGUEL A. F. SANJUÁN
Keyword(s):  
Gene Regulation ◽  
Genetic Networks ◽  
Nonlinear Circuits ◽  
Synthetic Gene ◽  
Electronic Circuits ◽  
Biochemical System ◽  
Gene Regulation Networks ◽  
Electronic Elements ◽  
Electronic Design

We propose the use of nonlinear electronic circuits to study synthetic gene regulation networks. Specifically, we have designed two electronic versions of a synthetic genetic clock, known as the "repressilator," making use of appropriate electronic elements linked in the same way as the original biochemical system. We study the effects of coupling in a population of electronic repressilators, with the aim of observing coherent oscillations of the whole population. With these results, we show that this kind of nonlinear circuits can be helpful in the design and understanding of synthetic genetic networks.

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