Results 141 to 150 of about 79,468 (267)

Proteomic Profiling of Myofiber Repair Annexins and Their Role in Duchenne Muscular Dystrophy

PROTEOMICS, EarlyView.
ABSTRACT Myofiber regeneration and membrane repair play crucial roles in maintaining the continuous physiological functioning of the neuromuscular system. A swift and efficient repair mechanism enables the rapid restoration of sarcolemmal integrity following cellular impairment in damaged skeletal muscles.
Paul Dowling, Dounia Bouragba, Elisa Negroni, Capucine Trollet, Margit Zweyer, Dieter Swandulla, Kay Ohlendieck   +6 more
wiley   +1 more source

A muscular dystrophy associated with bi‐allelic LEMD2 variants: Expanding the genotype of nuclear envelopathies

Brain Pathology, EarlyView.
Proteomics‐guided exome re‐analysis identifies bi‐allelic variants in the nuclear envelope LEMD2 gene, expanding its phenotypic spectrum. Created in BioRender. Pauper, M. (2026) https://BioRender.com/xamvo92.
Marc Pauper, Heike Kölbel, Iakowos Karakesisoglou, Anne Schänzer, Johann Böhm, Rachel Thompson, Nicolai Kohlschmidt, Bernd Ringel, Gisèle Bonne, Katja Neuhoff, Andrea Gangfuß, Ozge Aksel Kilicarslan, Sergi Beltran Agullo, Andreas Hentschel, Ulrike Schara‐Schmidt, Hanns Lochmüller, Kiran Polavarapu, Andreas Roos   +17 more
wiley   +1 more source

Protein kinase C activity modulates nuclear Lamin A/C dynamics in HeLa cells

Scientific Reports
The nuclear lamina serves important functions in the nucleus, providing structural support to the nuclear envelope and contributing to chromatin organization. The primary proteins that constitute the lamina are nuclear lamins whose functions are impacted
Chase C. Wesley, Dallin V. North, Daniel L. Levy   +2 more
doaj   +1 more source

ABHD7-mediated depalmitoylation of lamin A promotes myoblast differentiation

Cell Reports
Summary: LMNA gene mutation can cause muscular dystrophy, and post-translational modification plays a critical role in regulating its function. Here, we identify that lamin A is palmitoylated at cysteine 522, 588, and 591 residues, which are reversely ...
Yuan Shen, Liang-Liang Zheng, Cai-Yun Fang, Yao-Yao Xu, Chao Wang, Jin-Tao Li, Ming-Zhu Lei, Miao Yin, Hao-Jie Lu, Qun-Ying Lei, Jia Qu   +10 more
doaj   +1 more source

The metalloproteinase ADAM17 promotes acute lung inflammatory responses during pancreatitis

British Journal of Pharmacology, EarlyView.
Background and Purpose Acute pancreatitis (AP) is a multifactorial upper gastrointestinal inflammatory disorder that in severe cases (~20% of all AP) is associated with substantial morbidity and mortality, the latter coincident with multiorgan dysfunction, particularly acute lung injury (ALI).
Shermin Chan, Karen Hon, Ruby E. Dawson, Teresa Weng, Jelena Solujic, Yu C. J. Chey, James D. Zuiani, Griffith B. Perkins, P. Toby Coates, Chris Drogemuller, Eddie Sanderlin, Lulu Huang, John Finnie, Stefan Rose‐John, Arash Badiei, Phan Nguyen, Brendan J. Jenkins, Mohamed I. Saad   +17 more
wiley   +1 more source

LPC18:0 Secreted by Exogenous Neural Stem Cells Potentiates Neurogenesis and Functional Recovery via GPR55‐Mediated Signalling in Spinal Cord Injury

Cell Proliferation, EarlyView.
LPC18:0 secreted by exogenous neural stem cells potentiates neurogenesis via the GPR55/AKT/GSK3β signalling axis, which ultimately promotes spinal cord injury recovery. ABSTRACT Spinal cord injury (SCI) is a devastating condition with limited therapeutic options. Although neural stem cell (NSC) transplantation shows regenerative potential, its efficacy
Dong Chen, Shuo Liu, Le‐Yi Tu, Ming‐Mei Yang, Cong‐Wang Xu, Yue Jiang, Hui Yang, Chen‐Xu Tai, Yan‐Ning Wang, Yuan‐Yuan Xie, Ping‐Ping Shen, Bin Wang   +11 more
wiley   +1 more source

Heat Stress Triggers Nuclear Invagination and Spatial Compartmentalization of Protein Metabolism

Cell Proliferation, EarlyView.
Cells adapt heat stress to shape a nuclear invagination region function as “protein metabolism hotspots”, where both protein production and degradation are enhanced. ABSTRACT Heat stress is a common challenge for cells, causing multiple types of cellular damage while triggering complex stress responses, including the highly conserved mechanism known as
Zhi‐Hao Zhang, Yuan‐Tao Qiu, Jing‐Yue Cao, Yi‐Feng Huang, Ye‐Lin Lan, Yu‐Li Zhao, Fu Wu, Chen‐Long Wang, Guo‐Run Tang, Jing‐Yun Ji, Zhang Zhang, Guan‐Zheng Luo   +11 more
wiley   +1 more source

Proteostasis of organelles in aging and disease

The FEBS Journal, EarlyView.
Cells rely on regulated proteostasis mechanisms to keep their internal compartments functioning properly. When these mechanisms fail, damaged proteins accumulate, disrupting organelles, such as the nucleus, mitochondria, endoplasmic reticulum, Golgi, and lysosomes, as well as membraneless organelles, such as stress granules, processing bodies, the ...
Yara Nabawi, Cansu Doğan, Dunja Petrović, Gülce Perçin, Seda Koyuncu, David Vilchez   +5 more
wiley   +1 more source

A large genomic deletion leads to enhancer adoption by the lamin B1 gene: a second path to autosomal dominant adult-onset demyelinating leukodystrophy (ADLD) [PDF]

, 2017
Chromosomal rearrangements with duplication of the lamin B1 (LMNB1) gene underlie autosomal dominant adult-onset demyelinating leukodystrophy (ADLD), a rare neurological disorder in which overexpression of LMNB1 causes progressive central nervous system ...
Antonarakis, Stylianos E., Atzori, Cristiana, Brusco, Alfredo, Brussino, Alessandro, Canale, Claudio, Di Gregorio, Eleonora, Ferrera, Denise, Ferrero, Enza, Gasparini, Laura, Giorgio, Elisa, Guipponi, Michel, Imperiale, Daniele, Pennacchio, Len A., Robyr, Daniel, Santoni, Federico, Spielmann, Malte, Stamoulis, Georgios, Vaula, Giovanna   +17 more
core  

Resistance‐exercise‐induced stress intervenes in TGF‐β signaling by cooperatively downregulating nuclear αB‐crystallin and SMAD4 in human skeletal muscle fibers

The FEBS Journal, EarlyView.
Alpha‐crystallin B chain (CRYAB) has been reported to stabilize mothers against decapentaplegic homolog 4 (SMAD4) in transforming growth factor‐β (TGF‐β) signaling, enabling target gene transcription. We show nuclear CRYAB–SMAD4 interaction for the first time in human skeletal muscle fibers and its regulation by exercise.
Kirill Schaaf, Cassandra Duda, Tihomir Kostov, Patrick Diel, Käthe Bersiner, Wilhelm Bloch, Sebastian Gehlert, Daniel Jacko   +7 more
wiley   +1 more source