Výsledky vyhledávání - "dystrophin-associated glycoprotein complex"

Akademický článek

Incomplete Assembly of the Dystrophin-Associated Protein Complex in 2D and 3D-Cultured Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes

Autor: Guillaume Gilbert, Chandan Kadur Nagaraju, Robin Duelen, Matthew Amoni, Pierre Bobin, Thomas Eschenhagen, H. Llewelyn Roderick, Maurilio Sampaolesi, Karin R. Sipido

Publikováno v: Frontiers in Cell and Developmental Biology, Vol 9 (2021)

Human induced pluripotent stem cells derived cardiomyocytes (hiPSC-CM) are increasingly used to study genetic diseases on a human background. However, the lack of a fully mature adult cardiomyocyte phenotype of hiPSC-CM may be limiting the scope of t

Externí odkaz: https://doaj.org/article/b35c150cb7c84d46b27ae8cd2dd0d249

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Interrogation of Dystrophin and Dystroglycan Complex Protein Turnover After Exon Skipping Therapy

Autor: Christopher B. Tully, Terence A. Partridge, Utkarsh J. Dang, Ravi Hindupur, Davi A. G. Mázala, James S. Novak, Kristy J. Brown, Yetrib Hathout, Emily H. Canessa, Alyson A. Fiorillo, Kanneboyina Nagaraju, Rita Spathis

Publikováno v: Journal of Neuromuscular Diseases

Recently, the Food and Drug Administration granted accelerated approvals for four exon skipping therapies –Eteplirsen, Golodirsen, Viltolarsen, and Casimersen –for Duchenne Muscular Dystrophy (DMD). However, these treatments have only demonstrate

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::aeeee445cf10ae357bc01250c19e86ef
https://doi.org/10.3233/jnd-210696

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Akademický článek

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Increasing LRP4 diminishes neuromuscular deficits in a mouse model of Duchenne muscular dystrophy

Autor: Hongyang Jing, Tiankun Hui, Peng Chen, Shunqi Wang, Daojun Hong, Min Yan, Tian Zhou, Xia Dong, Suqi Zou, Ziyang Liu, Erkang Fei, Dongyan Ren, Xinsheng Lai

Publikováno v: Human Molecular Genetics

Duchenne muscular dystrophy (DMD) is an X-linked neuromuscular disease characterized by progressive wasting of skeletal muscles. The neuromuscular junction (NMJ) is a synapse between motor neurons and skeletal muscle fibers, critical for the control

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::6cb76b0f25b4cc642bcc68e6b0e98618
http://europepmc.org/articles/PMC8369839

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Duchenne muscular dystrophy cell culture models created by CRISPR/Cas9 gene editing and their application in drug screening

Autor: E. Ruiz-Del-Yerro, Aina Anton-Martinez, Javier Poyatos-Garcia, Edurne Albiasu-Arteta, Laura de la Puente-Ovejero, Gabriela Gonzalez-Iglesias, Patricia Soblechero-Martín, Virginia Arechavala-Gomeza, I. Garcia-Jimenez, Irene Larranaga-Aiestaran, Federico Gonzalez, Andrea López-Martínez

Publikováno v: Scientific Reports, Vol 11, Iss 1, Pp 1-14 (2021)
Scientific Reports
SCIENTIFIC REPORTS
r-IIS La Fe. Repositorio Institucional de Producción Científica del Instituto de Investigación Sanitaria La Fe
instname

Gene editing methods are an attractive therapeutic option for Duchenne muscular dystrophy, and they have an immediate application in the generation of research models. To generate myoblast cultures that could be useful in in vitro drug screening, we

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::ec490975a384fc3fca7b2fbba35f0d67
https://doi.org/10.1038/s41598-021-97730-5

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P–062 Annexin-V MACS method could be a good choice for sperm selection with high PLCZ1 expression and high blastocyst rate in high DFI male factor

Autor: M Saleh. Novin, M Bakhtiari, Reza Aflatoonian, Z Zandie

Publikováno v: Human Reproduction. 36

Study question What effect does Annexin-V MACS method have on sperm selection by pLCZ1 expression and blastocyst rate in male DNA fragmentation? Summary answer AnnexinV-MACS method could be a good choice for sperm selection with high pLCZ1 expression

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::b782cb7dc1105e85fb43d9c2f690eafb
https://doi.org/10.1093/humrep/deab130.061

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Circuit-based interventions in the dentate gyrus rescue epilepsy-associated cognitive dysfunction

Autor: Hajime Takano, Cuiyong Yue, Russell G. Port, Douglas A. Coulter, Julia B. Kahn

Publikováno v: Brain

Temporal lobe epilepsy is associated with significant structural pathology in the hippocampus. In the dentate gyrus, the summative effect of these pathologies is massive hyperexcitability in the granule cells, generating both increased seizure suscep

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d4b04c8b2c260f40b940fda032316ddc
https://doi.org/10.1093/brain/awz209

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TheTreponema denticolaDgcA protein (TDE0125) is a functional diguanylate cyclase

Autor: Richard T. Marconi, Edward J A Schuler, Dhara T Patel, Nathaniel S. O'Bier

Publikováno v: Pathogens and Disease. 79

Periodontal disease (PD) is a progressive inflammatory condition characterized by degradation of the gingival epithelium, periodontal ligament, and alveolar bone ultimately resulting in tooth loss. Treponema denticola is a keystone periopathogen that

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::1e868ab8ccb12291d1d8efc376a1d14e
https://doi.org/10.1093/femspd/ftab004

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Loss of sarcospan exacerbates pathology in mdx mice, but does not affect utrophin amelioration of disease

Autor: Katherine G. Hammond, Elizabeth M. Gibbs, Kara M Shin, Jamie L. Marshall, Jackie L McCourt, Rachelle H. Crosbie

Publikováno v: Hum Mol Genet

The dystrophin–glycoprotein complex (DGC) is a membrane adhesion complex that provides structural stability at the sarcolemma by linking the myocyte’s internal cytoskeleton and external extracellular matrix. In Duchenne muscular dystrophy (DMD),

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::1651f2d41e93d82b0887ee001bb81060
https://europepmc.org/articles/PMC8091037/

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Natural products, PGC-1 alpha, and Duchenne muscular dystrophy

Autor: Seyed Mohammad Nabavi, Samira Shirooie, Rosa Anna Vacca, Ipek Süntar, Tarun Belwal, Ana Sanches Silva, Antoni Sureda, Maryam Majidinia, Suowen Xu, Giuseppe D'Antona, Ahmad Reza Dehpour, Seyed Fazel Nabavi, Devesh Tewari, Eduardo Sobarzo-Sánchez, Bahman Yousefi, Maria Daglia

Publikováno v: Acta Pharmaceutica Sinica B, Vol 10, Iss 5, Pp 734-745 (2020)
Acta pharmaceutica sinica B
info:cnr-pdr/source/autori:Suntar I.; Sureda A.; Belwal T.; Sanches Silva A.; Vacca R.A.; Tewari D.; Sobarzo-Sanchez E.; Nabavi S.F.; Shirooie S.; Dehpour A.R.; Xu S.; Yousefi B.; Majidinia M.; Daglia M.; D'Antona G.; Nabavi S.M./titolo:Natural products, PGC-1alpha, and Duchenne muscular dystrophy/doi:10.1016%2Fj.apsb.2020.01.001/rivista:Acta pharmaceutica sinica B (Print)/anno:2020/pagina_da:734/pagina_a:745/intervallo_pagine:734–745/volume:10
Acta Pharmaceutica Sinica. B

Peroxisome proliferator-activated receptor gamma (PPAR gamma) is a transcriptional coactivator that binds to a diverse range of transcription factors. PPAR gamma coactivator 1 (PGC-1) coactivators possess an extensive range of biological effects in d

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9e63841db77843060607354e31e90f64
https://hdl.handle.net/20.500.13003/17243

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