Recessive mutant gene c in axolotl embryos results in an absence of normal heart function. Immunofluorescence studies were done to determine the distributions of myosin, tropomyosin and alpha-actinin in the hearts of normal and mutant siblings. Anti-myosin specifically stains the A bands of myofibrils in normal hearts and reveals a progressive increase in myofibril organization with development. Mutant hearts display less staining for myosin than normal and localization is mainly in amorphous collections. Anti-alpha-actinin stains the Z lines of myofibrils in normal myocytes. Mutant cells also have significant staining for alpha-actinin but show no striations. Antitropomyosin intensely stains the I bands of myofibrils in normal cells; however, there is very little staining for tropomyosin in mutant hearts. Thus, mutant myocardial cells have reduced but significant amounts of actin (Lemanski, Mooseker, Peachey & Iyengar, 1976) and myosin, even though non-filamentous, and substantial amounts of alpha-actinin. The cells appear to contain little tropomyosin.
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Vet Clin North Am Equine Pract
January 2025
Department of Clinical Sciences, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins, CO, USA. Electronic address:
Myofibrillar myopathy (MFM) is characterized by segmental disarray of myofibrils and ectopic accumulation of a protein called desmin. Previously thought to be a glycogen storage disease, MFM is now recognized as a stand-alone myopathy. Endurance Arabians with MFM usually present with exertional rhabdomyolysis (MFM-ER) at the end of races, elevated serum muscle enzymes, and myoglobinuria.
View Article and Find Full Text PDFCytoskeleton (Hoboken)
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Department of Biochemistry and Molecular Biophysics, Kansas State University, Manhattan, Kansas, USA.
Muscle development and maintenance is central to the normal functioning of animals. Muscle tissues exhibit high levels of activity and require the dynamic turnover of proteins. An actomyosin scaffold functions with additional proteins comprising the basic contractile subunit of striated muscle, known as the sarcomere.
View Article and Find Full Text PDFSci Rep
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Department of Congenital Heart Defects and Pediatric Cardiology, German Heart Center Munich, TUM University Hospital, School of Medicine & Health, Technical University of Munich, Munich, Germany.
Hypertrophic cardiomyopathy (HCM) caused by autosomal-dominant mutations in genes coding for structural sarcomeric proteins, is the most common inherited heart disease. HCM is associated with myocardial hypertrophy, fibrosis and ventricular dysfunction. Hypoxia-inducible transcription factor-1α (Hif-1α) is the central master regulators of cellular hypoxia response and associated with HCM.
View Article and Find Full Text PDFFASEB J
January 2025
Department of Biochemistry and Molecular Biology, Institute of Marine and Environmental Technology, University of Maryland School of Medicine, Baltimore, Maryland, USA.
Objective: Aim: To determine the profound inf l uence of scorpion venom toxins on the intricate structure of the heart of mammals, a topic of utmost importance in toxicology and cardiovascular health.
Patients And Methods: Materials and Methods: A meticulous and comprehensive literature analysis was conducted using PubMed, Google Scholar, Web of Science, and Scopus databases. We meticulously selected the newest publications up to 5 years old or the most thorough publications that vividly described our topic's essence, ensuring our findings' credibility and reliability.
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