• Drosophila melanogaster , the fruit fly, expresses both forms of striated muscle and benefits greatly from powerful genetic tools. (hopkinsmedicine.org)
  • Here, we propose to use a transgenic animal model system, Drosophila melanogaster (the fruit fly), to define the mechanisms by which mutations in various thin filament components lead to human cardiac disease. (hopkinsmedicine.org)
  • Using a transgenic model system, the fruit fly Drosophila melanogaster, we will produce models of human myosin-based muscle disease (distal arthrogryposis) and heart disease (hypertrophic cardiomyopathy) to determine the molecular defects that cause abnormal skeletal and cardiac muscle function. (hopkinsmedicine.org)
  • In wild-type embryos of Drosophila melanogaster , the formation of differentiated larval muscles is preceded by the segregation of small numbers of progenitor or founder cells in the embryonic mesoderm. (biologists.com)
  • After acute injury in muscle of both mouse and the fruit fly Drosophila melanogaster (used as alternative in vivo model) this study found that RACK1 accumulated in regenerating fibers while it declined with the progression of repair process. (sdbonline.org)
  • With the examples of synaptic active zones, centrosomes and sarcomeres, we will explain the instrumental role of super-resolution imaging pioneered in Drosophila in understanding fundamental subcellular constituents. (bvsalud.org)
  • The contractile forces are generated by highly regular mini-machines called sarcomeres, which are organized in long periodic chains called myofibrils. (muscledynamics.org)
  • Using the fly model, the Schnorrer lab developed the tension-driven self-organization hypothesis of myofibrillogenesis, which suggests that mechanical tension acts as a compass to coordinate the assembly of many sarcomeres into long myofibrils. (muscledynamics.org)
  • In Drosophila with depleted RACK1 in all muscle cells or, specifically, in SC lineage resulted in a delayed recovery of skeletal muscle after physical damage as well as the low presence of active SC in the wound area. (sdbonline.org)
  • Defining the transcriptional mechanisms of heart and skeletal muscle development and disease, using Drosophila as the model system. (sdsu.edu)
  • Additional analysis demonstrates that kettin in myofibrils is attached to both actin and the myosin thick filament, and suggests that kettin and projectin are the major constituents of the elastic-filament system. (rupress.org)
  • We are using an integrative approach to study the structure-function relationship of the myosin SH1 domain in the Drosophila model system We constructed a gene encoding myosin with the single amino acid mutation and expressed it in place of wild-type myosin heavy chain by germline transformation and crossing into a line that lacks myosin in its flight and jump muscles. (sdsu.edu)
  • Our results suggest that the E699K mutation (corresponding the E706K in humans) in the SH1 helix of the myosin head severely affects myofibril structure and function in homozygous flies. (sdsu.edu)
  • These results suggest that one copy of E699K with one copy of wild-type Mhc in the Mhc__ background affects myosin function but not IFM myofibril assembly and structure. (sdsu.edu)
  • The goal of this project is to examine how specific intercalated disc proteins, which are upregulated with age as a part of a genotype, alter the age-associated performance and mechanical stiffness of the Drosophila heart tube. (hopkinsmedicine.org)
  • page 1045 ) now provide strong evidence that kettin is functionally equivalent to titin, and suggest that this protein and projectin are the main contributors to the high stiffness of myofibrils in insects. (rupress.org)
  • Using a novel technique to analyze the mechanical properties of individual myofibrils from Drosophila indirect flight muscle, the authors show that disrupting kettin immediately decreases myofibril stiffness. (rupress.org)
  • Large kettin isoforms are associated with muscle tissues that are more extensible and less stiff, leading the authors to propose that insect myofibril stiffness is determined by the specific isoforms of projectin and kettin expressed in different muscle types. (rupress.org)
  • 2) Investigate the effect of aging on passive mechanical properties of Drosophila myocardium with cardiac-specific overexpression of the transcription factor FOXO, which is known to rejuvenate cardiac performance and promote muscle proteostasis in senescent flies. (hopkinsmedicine.org)
  • Knockdown phenotypes of spliceosome components, as highlighted by phenotypes for A-complex components SF1 and Hrb87F (hnRNPA1), revealed level- and temporal-dependent myofibril defects. (uni-muenchen.de)
  • Our results expand the number of RBPs with a described phenotype in muscle and underscore the diversity in myofibril and transcriptomic phenotypes associated with splicing defects. (uni-muenchen.de)
  • The Schnorrer lab pioneers the interface between developmental and cell biology with biophysics in order to understand how the ordered sarcomeric machine is assembled in Drosophila and human muscle models. (muscledynamics.org)
  • The Hippo pathway controls myofibril assembly and muscle fiber growth by regulating sarcomeric gene expression. (mpg.de)
  • The repeating contractile units of the MYOFIBRIL, delimited by Z bands along its length. (lookformedical.com)
  • We further show that splicing mediated by SF1 and Hrb87F is necessary for Z-disc stability and proper myofibril development, and strong knockdown of either gene results in impaired localization of kettin to the Z-disc. (uni-muenchen.de)
  • How can such long and regular myofibrils form during development? (muscledynamics.org)
  • In vertebrates this subfamily contains four proteins: TIF1α/TRIM24, TIF1β/TRIM28, TIF1γ/TRIM33, and TIF1δ/TRIM66, while only one protein, Bonus (Bon), is present in Drosophila , making it an attractive model to understand the conserved functions of TIF1 proteins. (elifesciences.org)
  • Here, we demonstrate the utility of Drosophila as a genetic model system to investigate basic developmental mechanisms of RBP function in myogenesis. (uni-muenchen.de)
  • This study aims to understand the function and mechanism of Drosophila Glycine N-acyltransferase (GLYAT) in cell invasion. (sdbonline.org)
  • Here, we summarize the major nanoscopic techniques and illustrate how these approaches were used in Drosophila model systems to revisit a series of well-known cell biological phenomena. (bvsalud.org)
  • In the heterozygous condition, the myofibril structure shows normal thick and thin filament packing in young and older flies. (sdsu.edu)
  • In myocardial cells, aII spectrin distribution is coincident with Z-discs and the plasma membrane of myofibrils. (wikipedia.org)
  • Insight into insulin secretion from transcriptome and genetic analysis of insulin-producing cells of Drosophila. (spletterlab.com)
  • The levels of catalase (CAT) and glutathione (GSH) were measured in PC12 cells and Drosophila brain tissue. (sdbonline.org)
  • GSOs also mitigated the deleterious effects of GLU on the mitochondrial membrane potential and Cyt C release, thus alleviating mitochondrial dysfunction, and increased GSH levels and CAT activity in both cells and Drosophila brain tissue. (sdbonline.org)
  • The RNA-binding protein Arrest (Bruno) regulates alternative splicing to enable myofibril maturation in Drosophila flight muscle. (spletterlab.com)
  • Tension and Force-Resistant Attachment Are Essential for Myofibrillogenesis in Drosophila Flight Muscle. (mpg.de)
  • This approach will directly test how defined forces and shapes instruct myofibrillogenesis in human muscle, and will thus generalize the tension-driven myofibril self-organization model to humans. (muscledynamics.org)
  • Here, we report structures of Drosophila Slo in the Ca2+-bound and Ca2+-free form and in complex with the fungal neurotoxin verruculogen and the anthelmintic drug emodepside. (bvsalud.org)
  • This important study advances our understanding of the functions and regulation of the Drosophila transcriptional regulator Bonus, an ortholog of mammalian TIF1 family members. (elifesciences.org)
  • The specific aims of the project are: 1) Identify differences in potential collective, age-related myocardial stiffening events among multiple Drosophila control lines. (hopkinsmedicine.org)
  • The specific aims of the project are: 1) Identify age-related changes in cardiac expression profiles using GeneChip microarrays that represent the complete Drosophila genome. (hopkinsmedicine.org)