Results 31 to 40 of about 4,236 (177)

C-terminal tagging, transmembrane domain hydrophobicity, and an ER retention motif influence the secretory trafficking of the inner nuclear membrane protein emerin [PDF]

eLife
The inner nuclear membrane (INM), a subdomain of the endoplasmic reticulum (ER), sequesters hundreds of transmembrane proteins within the nucleus. We previously found that one INM protein, emerin, can evade the INM by secretory transport to the lysosome,
Jessica Mella, Regan F Volk, Balyn W Zaro, Abigail Buchwalter   +3 more
doaj   +2 more sources

Profibrotic Molecules Are Reduced in CRISPR-Edited Emery–Dreifuss Muscular Dystrophy Fibroblasts [PDF]

Cells
Emery–Dreifuss muscular dystrophy (EDMD) is caused by mutations in EMD, LMNA, SYNE1, SYNE2, and other related genes. The disease is characterized by joint contractures, muscle weakening and wasting, and heart conduction defects associated with dilated ...
Eleonora Cattin, Elisa Schena, Elisabetta Mattioli, Stefania Marcuzzo, Silvia Bonanno, Paola Cavalcante, Federico Corradi, Daniela Benati, Giorgia Farinazzo, Marco Cattaneo, Veronica De Sanctis, Roberto Bertorelli, Lorenzo Maggi, Melania Giannotta, Antonella Pini, Gaetano Vattemi, Denise Cassandrini, Marco Cavallo, Cristina Manferdini, Gina Lisignoli, Beatrice Fontana, Ilaria Pace, Claudio Bruno, Roberta Roncarati, Chiara Fiorillo, Manuela Ferracin, Eric C. Schirmer, Alessandra Recchia, Giovanna Lattanzi   +28 more
doaj   +2 more sources

Essential Role for Telomeric Repeat-Binding Factor 2 in Cardiac Development and Function. [PDF]

FASEB Bioadv
This schematic illustrates a paradigm shift in telomere biology, demonstrating that Trf2 is a mandatory orchestrator of heart development and function through pathways distinct from its canonical telomere protective role. ABSTRACT Telomere repeat‐binding factor 2 (Trf2) is essential for protecting our telomeres.
Hakim Shoushtari A, Danis A, Stoner MW, Baust J, Sebastiani A, Bugga P, Manning JR, Mushala BAS, Bhattarai N, Al Ghouleh I, Scott I, Sharifi-Sanjani M.   +11 more
europepmc   +2 more sources

A-type lamins anchor emerin at the inner nuclear membrane via two independent binding sites. [PDF]

J Biol Chem
Lamins form a dense meshwork at the inner surface of the inner nuclear membrane (INM), where they interact with other nuclear envelope proteins such as emerin.
Odell J, Nedza K, Lammerding J.
europepmc   +2 more sources

The Role of Emerin in Cancer Progression and Metastasis [PDF]

International Journal of Molecular Sciences, 2021
It is commonly recognized in the field that cancer cells exhibit changes in the size and shape of their nuclei. These features often serve as important biomarkers in the diagnosis and prognosis of cancer patients. Nuclear size can significantly impact cell migration due to its incredibly large size.
Alexandra G. Liddane, James M. Holaska
openaire   +2 more sources

Current Topics of Progressive Cardiac Conduction Disease. [PDF]

J Arrhythm
Many genes and the protein cause PCCD. Mutation of NaV1.5 or CX40 cause isolated PCCD, but mutation of lamin A/C, emerin, or desmin lead to cardiomyopathy, and PCCD. Mutation of transcription factor NCX2‐5, and Tbx5 associated with atrial septal defect and abnormal development of conduction system.
Sumitomo N, Mori H, Kumamoto T, Ishikawa T, Seki A, Makita N, Schott JJ, Wilde AAM.   +7 more
europepmc   +2 more sources

Role of Emerin in regulating fibroblast differentiation and migration at the substrate of stiffness coupled topology

Acta Biochimica et Biophysica Sinica
In hypertrophic scars, the differentiation and migration of fibroblasts are influenced by the extracellular matrix microenvironment, which includes factors such as stiffness, restraint, and tensile force.
Yang Tiantian, Wang Li, Ma Haiyang, Li Kailun, Wang Yajing, Tang Wenjie, Wang Zichen, An Meiwen, Gao Xiang, Xu Ludan, Guo Yunyun, Guo Jiqiang, Liu Yong, Wang Hugen, Liu Yang, Zhang Quanyou   +15 more
doaj   +2 more sources

Lmo7 is an emerin-binding protein that regulates the transcription of emerin and many other muscle-relevant genes [PDF]

Human Molecular Genetics, 2006
X-linked Emery-Dreifuss muscular dystrophy (X-EDMD) is inherited through mutations in emerin, a nuclear membrane protein. Emerin has proposed roles in nuclear architecture and gene regulation, but direct molecular links to disease were unknown. We report that Lim-domain only 7 (Lmo7) binds emerin directly with 125 nM affinity; the C-terminal half of ...
James M, Holaska, Soroush, Rais-Bahrami, Katherine L, Wilson   +2 more
openaire   +2 more sources

Loss of emerin alters myogenic signaling and miRNA expression in mouse myogenic progenitors. [PDF]

PLoS ONE, 2012
Emerin is an integral membrane protein of the inner nuclear membrane. Mutations in emerin cause X-linked Emery-Dreifuss muscular dystrophy (EDMD), a disease characterized by skeletal muscle wasting and dilated cardiomyopathy.
Adam J Koch, James M Holaska
doaj   +1 more source

Identification of PP2A-B55 targets uncovers regulation of emerin during nuclear envelope reassembly in Drosophila

Open Biology, 2023
Mitotic exit requires the dephosphorylation of many proteins whose phosphorylation was needed for mitosis. Protein phosphatase 2A with its B55 regulatory subunit (PP2A-B55) promotes this transition.
Virginie Emond-Fraser, Myreille Larouche, Peter Kubiniok, Éric Bonneil, Jingjing Li, Mohammed Bourouh, Laura Frizzi, Pierre Thibault, Vincent Archambault   +8 more
doaj   +1 more source