Postnatal centralization of muscle fibre nuclei, which were previously located subsarcolemmally, is described in a case of centronuclear myopathy (CNM) in a male patient with generalized muscle weakness since birth. A muscle biopsy was taken at the age of 11 months; no particular abnormalities were observed at this stage apart from an unusual variation in fibre size. A distinctly below average muscle fibre diameter, increased endomysial connective tissue, and features typical for CNM were found in a biopsy taken 9 yr later. Immunohistochemical studies using antibodies to desmin, vimentin, laminin and type IV collagen revealed altered staining patterns compared with normal fibres. The abnormalities in the patterns of cytoskeletal proteins point to a defective regulation of the composition and organization of the cytoskeletal network during development, paralleled by abnormalities in the extracellular matrix.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/0960-8966(91)90027-p | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Photobiomodulation and aquatic training reduce TNF-α expression and enhance muscle fiber area in Wistar rats with compensatory hypertrophy.
Lasers Med Sci
January 2025
Postgraduate Program in Rehabilitation Sciences, Universidade Nove de Julho (UNINOVE), 235/249 Vergueiro Street, Sao Paulo, SP, 01525000, Brazil.
This study aims to assess the effects of aquatic training (AT) and its combination with photobiomodulation (PBM) on cytokine synthesis and plantar muscle morphology during compensatory hypertrophy (H) in Wistar rats. H was induced by bilateral ablation of synergistic muscles, and PBM using a laser (780 nm). AT involved 60 min sessions, 5 times/week, for 7 and 14 days.
View Article and Find Full Text PDFTroponins and Skeletal Muscle Pathologies.
Biochemistry (Mosc)
December 2024
Faculty of Biology, Lomonosov Moscow State University, Moscow, 119234, Russia.
Skeletal muscles account for ~30-40% of the total weight of human body and are responsible for its most important functions, including movement, respiration, thermogenesis, and glucose and protein metabolism. Skeletal muscle damage negatively impacts the whole-body functioning, leading to deterioration of the quality of life and, in severe cases, death. Therefore, timely diagnosis and therapy for skeletal muscle dysfunction are important goals of modern medicine.
View Article and Find Full Text PDFWater-regulated viscosity-plasticity phase transitions in a peptide self-assembled muscle-like hydrogel.
Nat Commun
January 2025
Department of Chemistry, School of Science, Westlake University, Hangzhou, Zhejiang Province, China.
The self-assembly of small molecules through non-covalent interactions is an emerging and promising strategy for building dynamic, stable, and large-scale structures. One remaining challenge is making the non-covalent interactions occur in the ideal positions to generate strength comparable to that of covalent bonds. This work shows that small molecule YAWF can self-assemble into a liquid-crystal hydrogel (LCH), the mechanical properties of which could be controlled by water.
View Article and Find Full Text PDFSpatiotemporal metabolic mapping of ex-situ preserved hearts subjected to dialysis by integration of bio-SPME sampling with non-targeted metabolipidomic profiling.
Anal Chim Acta
February 2025
Department of Chemistry, University of Waterloo, Waterloo, ON, Canada. Electronic address:
Background: Normothermic ex situ heart perfusion (ESHP) has emerged as a valid modality for advanced cardiac allograft preservation and conditioning prior to transplantation though myocardial function declines gradually during ESHP thus limiting its potential for expanding the donor pool. Recently, the utilization of dialysis has been shown to preserve myocardial and coronary vasomotor function. Herein, we sought to determine the changes in myocardial metabolism that could support this improvement.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!