Results 91 to 100 of about 30,048 (267)
Sarcomere Dysfunction in Nemaline Myopathy
Journal of Neuromuscular Diseases, 2017 Nemaline myopathy (NM) is among the most common non-dystrophic congenital myopathies (incidence 1:50.000). Hallmark features of NM are skeletal muscle weakness and the presence of nemaline bodies in the muscle fiber. The clinical phenotype of NM patients is quite diverse, ranging from neonatal death to normal lifespan with almost normal motor function.
de Winter, Josine M +1 more
openaire +5 more sources
Advanced Science, EarlyView.We present the MyoRobot 3.0, an advanced platform that combines high‐resolution imaging with automated mechanical testing to improve the accuracy of single muscle fibre analysis. The system tracks fibre diameter changes during stretch, preventing major stress miscalculations.
Michael Haug +5 more
wiley +1 more source
Advanced Science, EarlyView.ABSTRACT Doxorubicin‐induced cardiomyopathy (DIC) remains a dose‐limiting clinical challenge. This study reveals that cardiac vascular endothelial cells (CVECs) act as initial sensors of doxorubicin cardiotoxicity: circulating doxorubicin activates the cGAS‑STING pathway in CVECs, triggering NLRP3 inflammasome‑mediated pyroptosis and release of ...
Wang Jun +10 more
wiley +1 more source
SMYD2 glutathionylation contributes to degradation of sarcomeric proteins
Nature Communications, 2018 Ischemic reperfusion or nutrient deprivation that produces reactive oxygen species can lead to a loss of muscle contractile function. Here the authors show that glutathionylation of the lysine methyltransferase SMYD2 contributes to degradation or ...
Dhanushka N. P. Munkanatta Godage +9 more
doaj +1 more source
Advanced Science, EarlyView.Vascular Aβ40 corrupts GRP78 phase behavior in brain endothelial cells, sustaining IRE1α–TRAF2–JNK signaling and driving apoptosis, tight junction loss, and blood–brain barrier failure in cerebral amyloid angiopathy. Pharmacological IRE1α inhibition restores vascular integrity, reduces leakage, and improves functional outcomes, revealing a targetable ...
Honglin Zheng +19 more
wiley +1 more source
Nature Communications, 2018 Protein phosphatase 5 (PP5) is expressed in many cell types but its role in cardiomyocytes is unknown. Here the authors show that PP5 binds and dephosphorylates elastic titin in cardiac sarcomeres, and that PP5 is increased in heart failure, reducing ...
Judith Krysiak +10 more
doaj +1 more source
A Novel Pak1 Activator Ameliorates ER Stress for HFpEF Therapy
Advanced Science, EarlyView.Chronic metabolic stress is a major contributor to HFpEF progression. Under prolonged metabolic stress, Pak1 activity becomes impaired, contributing to disrupted ER proteostasis, cardiomyocyte apoptosis, fibrosis, and diastolic dysfunction. Mechanistically, Pak1 overexpression activates the ERK1/2–MNK1–eIF4E signaling axis, promotes translational ...
Honglin Xu +17 more
wiley +1 more source
Sarcomeric proteins and inherited cardiomyopathies [PDF]
Cardiovascular Research, 2007 Over the last two decades, a large number of mutations have been identified in sarcomeric proteins as a cause of hypertrophic, dilated or restrictive cardiomyopathy. Functional analyses of mutant proteins in vitro have revealed several important functional changes in sarcomeric proteins that might be primarily involved in the pathogenesis of each ...
openaire +2 more sources
The Anatomical Record, EarlyView.Abstract Muscle architecture is a major determinant of muscle performance and, in mammalian lineages, has been correlated with both feeding ecology and locomotor behaviors. Over the past decade, contrast‐enhanced micro‐CT (DiceCT) has emerged as an alternative to traditional dissection‐based measurement.
Aleksandra Ratkiewicz +5 more
wiley +1 more source
Three‐dimensional digital analysis of musteloid masticatory muscle architecture
The Anatomical Record, EarlyView.Musteloids span an enormous breadth of dietary modes within a single superfamily. In this study, we utilize digital dissection techniques to reconstruct the internal 3D anatomy of muscles within the musteloid feeding apparatus, and explore how dietary specializations might alter the size, orientation, and curvature of fascicles therein.
Cassidy E. Davis +6 more
wiley +1 more source