• Plectin is a giant protein found in nearly all mammalian cells which acts as a link between the three main components of the cytoskeleton: actin microfilaments, microtubules and intermediate filaments. (wikipedia.org)
  • At the opposite end of the protein, in the N-terminal domain, a region has been defined as responsible for binding to actin. (wikipedia.org)
  • Ervasti, J.M. & Campbell, K.P. A role for the dystrophin-glycoprotein complex as a transmembrane linker between laminin and actin. (nature.com)
  • The role of the dystrophin protein is to anchor cytoskeleton of cardiac and skeletal muscle cells to the extracellular matrix, linking actin filaments to extracellular laminins. (fsahq.org)
  • Histopathologic changes were evaluated in medial gastrocnemius muscle samples using antibodies to cellular (actin, desmin, dystrophin, fast myosin) and extracellular (laminin, albumin, complement C3) proteins and markers for neutrophils (W3/13) and macrophages (ED1). (cdc.gov)
  • After 2 days, histopathologic changes were present only in samples from the S40 protocol including desmin negative fibers [162 (32)] which stained for albumin, complement C3, laminin, actin and fast myosin. (cdc.gov)
  • AAN02 is a mouse monoclonal antibody against actin protein. (cytoskeleton.com)
  • Actin is the major protein of the microfilament cytoskeletal system and is a key protein in various cell motility processes. (cytoskeleton.com)
  • The immunogen used for antibody production was purified actin protein from rabbit skeletal muscle. (cytoskeleton.com)
  • The antibody has been shown to recognize α-skeletal, α-cardiac, α-smooth muscle, β-cytoplasmic, γ-smooth muscle and γ- cytoplasmic actin isotypes (Fig. 1) and has broad species cross-reactivity. (cytoskeleton.com)
  • Dystrophin functions as a homotetramer at the costomeres in skeletal muscles, and it gets associated with actin at its N-terminus and the DAG complex at the C-terminus, forming a stable complex that interacts with laminin in the extracellular matrix. (ayurvedapc.blog)
  • Dystrophin associates with a number of proteins to form a large oligomeric complex named the dystrophin-glycoprotein complex (DGC), which bridges across the sarcolemma and connects the extracellular matrix and the actin cytoskeleton (Allamand and Campbell, (2000) Human Molecular Genetics 9:2459-2467). (justia.com)
  • Myofilaments are protein fibers consisting of thick ( myosin ) and thin ( actin ) filaments and are responsible for the contractile properties of muscle cells. (amboss.com)
  • Actin myofilaments are thin filaments composed of actin molecules, regulatory proteins , and nebulin. (amboss.com)
  • Regulatory proteins prevent permanent interaction between myosin and actin . (amboss.com)
  • Pel-Freez rabbit skeletal muscle actin aceton powder. (carlosvaldivieso.es)
  • Disruption of DAG1 in differentiated skeletal muscle reveals a role for dystroglycan in muscle regeneration. (nature.com)
  • Dystroglycan is a highly glycosylated extracellular matrix receptor with essential functions in skeletal muscle and the nervous system. (nature.com)
  • Dystroglycan is a core and essential component of the dystrophin-glycoprotein complex of muscle and brain, making vital links between the cytoskeleton and laminin in the extracellular matrix. (enmc.org)
  • Perturbations in the ability of dystroglycan to interact with laminin have severe implications for human disease. (enmc.org)
  • In rare, isolated instances they involve primary mutations of dystroglycan itself, but more frequently are associated with mutations in genes involved in the post-translational modification of dystroglycan: the secondary dystroglycanopathies, but also the so-called tertiary dystroglycanopathies arising from mutations in metabolic enzymes and other proteins that are involved more generally in multiple glycosylation pathways and cellular roles that in turn also impinge on dystroglycan function. (enmc.org)
  • Furthermore, detailed biochemical and cell biological analyses have finely mapped the biosynthetic pathways leading to the glycosylation of dystroglycan that enable it to bind to laminin. (enmc.org)
  • This wealth of information has led to a new understanding of how the defective interaction between of dystroglycan and laminin leads to disease. (enmc.org)
  • This allows dystroglycan to function as the predominant extracellular matrix receptor in skeletal muscle and brain. (enmc.org)
  • Moreover, in addition to our understanding of the glycosylation of dystroglycan, much insight has also been gained into the functional roles of dystroglycan, not only in the bran and muscle, but also in other cells types, and the implication that the defective processing of this protein has with respect to role in Lassa fever virus infections. (enmc.org)
  • Most LAMA2 gene mutations that cause the severe, early-onset form of LAMA2 -related muscular dystrophy result in the absence of functional laminin alpha-2 subunit. (medlineplus.gov)
  • Mutations that cause the milder, later-onset form usually result in a reduction (deficiency) of functional laminin alpha-2 subunit. (medlineplus.gov)
  • Mutations in LMX1B cause nail-patella syndrome (NPS), an autosomal dominant disease with skeletal abnormalities, nail hypoplasia, and nephropathy. (nih.gov)
  • Mutations in the gene coding for Fukutin related protein (FKRP) can lead to different diseases, ranging from very severe multi-organ syndromes like Walker Warburg Syndrome (WWS) and Muscle Eye Brain disease (MEB), to muscle defects as Congenital Muscle Dystrophy (CMD) and the mildest but more frequent form Limb Girdle Muscle Dystrophy type 2i (LGMD2i or LGMD-R9 FKRP-related). (mdaconference.org)
  • In muscular dystrophy, the problem is at the genomic level, where abnormal genes (mutations) interfere with the production of proteins needed to form healthy muscle tissue. (ayurvedapc.blog)
  • Hundreds of mutations in the genes that encode protein constituents of the sarcomere have been identified in HCM. (genome.jp)
  • I am particularly interested in the effects of mutations and polymorphisms on protein function since these studies can identify underlying pathologies and potential therapies. (cardiff.ac.uk)
  • PMID: 25486376 Mutations in Biosynthetic Enzymes for the Protein Linker Region of Chondroitin/Dermatan/Heparan Sulfate Cause Skeletal and Skin Dysplasias . (symptoma.com)
  • Cell motility measurements by computer-assisted time-lapse videomicroscopy of the alpha 7-transfected cells revealed an 8-10-fold increase in motility on laminin-1 and its E8 fragment, but not on fibronectin. (uea.ac.uk)
  • of muscles used for movement (skeletal muscles). (medlineplus.gov)
  • Laminin 2 and laminin 4 play a particularly important role in the skeletal muscles. (medlineplus.gov)
  • Prevention of histopathologic changes from 30 repeated stretches of active rat skeletal muscles by long inter-stretch rest times. (cdc.gov)
  • Multiple proteins are involved in the complex interactions for the proper functioning of the muscles. (ayurvedapc.blog)
  • The muscular dystrophies (MD) are a heterogeneous group of inherited disorders characterized by progressive weakness and degeneration of skeletal muscles. (justia.com)
  • The autoimmune attack occurs when autoantibodies form against the nicotinic acetylcholine postsynaptic receptors at the neuromuscular junction of skeletal muscles (see the image below). (medscape.com)
  • Laminin G-containing proteins appear to have a wide variety of roles in cell adhesion, signalling, migration, assembly and differentiation. (embl.de)
  • Biolaminin 221 supports the growth, survival, and differentiation of a wide range of tissue-specific cell types, including cardiac cells and skeletal muscle cells. (biolamina.com)
  • During myogenic differentiation, it may induce changes in the shape and mobility of myoblasts, and facilitate their localization at laminin-rich sites of secondary fiber formation. (cusabio.com)
  • Growth/differentiation factor-5 (GDF-5) belongs to the bone morphogenetic protein (BMP) family, which is expressed in dental pulp tissues. (ispub.com)
  • Growth/differentiation factor-5 (GDF-5) is a member of the bone morphogenetic protein (BMP) family, which is a subgroup of the transforming growth factor- (TGF-) superfamily. (ispub.com)
  • Using antibodies to podocyte proteins important for podocyte function, we found that Lmx1b(-/-) podocytes express near-normal levels of nephrin, synaptopodin, ZO-1, alpha3 integrin, and GBM laminins. (nih.gov)
  • Abcam: antibodies, proteins, kits. (abcam.com)
  • C) Inhibitory effects of anti-Lu/B-CAM antibodies on Lu-Fc binding to laminin α5. (bio-rad-antibodies.com)
  • This gene provides instructions for making a part (subunit) of certain members of a protein family called laminins. (medlineplus.gov)
  • Deficiency or absence of the laminin alpha-2 subunit results in a corresponding lack of laminin 2 and laminin 4, reducing the strength and stability of muscle tissue and leading to the signs and symptoms of LAMA2 -related muscular dystrophy. (medlineplus.gov)
  • The architecture of its extracellular domain is similar to that of neurexins, and this protein may be the signaling subunit of contactin, enabling recruitment and activation of intracellular signaling pathways in neurons. (nih.gov)
  • Integrin alpha-7/beta-1 is the primary laminin receptor on skeletal myoblasts and adult myofibers. (cusabio.com)
  • Acts as Schwann cell receptor for laminin-2. (cusabio.com)
  • [ 1 , 2 ] Although the chief target of the autoimmune attack in most cases is the skeletal muscle nicotinic acetylcholine receptor (nAChR), other antigenic targets that are components of the neuromuscular junction (NMJ) have also been implicated. (medscape.com)
  • Matriglycan is indeed a scaffold for multiple laminin-G (LG) domain-containing extracellular matrix proteins (e.g. laminin, agrin, and perlecan) and it has the remarkable capacity to be tuned during skeletal muscle development and regeneration. (enmc.org)
  • In cardiac muscle and skeletal muscle, plectin is localized to specialized entities known as Z-discs. (wikipedia.org)
  • Skeletal and cardiac muscle tissues were also significantly affected. (wikipedia.org)
  • In order to investigate and directly test the role of the alpha 7 integrin in cell migration on laminin, we expressed the murine alpha 7B splice variant in human 293 kidney cells and 530 melanoma cells which cannot migrate on laminin and are devoid of endogenous alpha 7. (uea.ac.uk)
  • Mouse anti Human CD239 antibody, clone BRIC221 ( MCA1982 ) used to look for blocking of Lu-Fc to endogenous α5 laminin in kidney sections. (bio-rad-antibodies.com)
  • Endogenous laminin α5 and bound Lu-Fc were detected with anti-laminin α5 domain LEb/L4b and anti-human IgG, as indicated. (bio-rad-antibodies.com)
  • Endogenous laminin α2 (upper panel) and α5 (lower panel) were detected with antisera. (bio-rad-antibodies.com)
  • The laminins attach (bind) to other proteins in the extracellular matrix and in the membrane of muscle cells, which helps maintain the stability of muscle fibers. (medlineplus.gov)
  • The absence of either alpha7beta1 integrin or alpha6beta1 integrin impairs the ability of Schwann cells to spread and to bind laminin. (cusabio.com)
  • Alpha-crystallins also act as molecular chaperones that bind to denatured proteins, keep them in solution and thereby maintain the translucency of the lens. (lookformedical.com)
  • To test whether complete removal of skeletal muscle tissue affects the spatiotemporal relationship between myogenesis and angiogenesis during regeneration, subthreshold volumetric muscle loss was created with a biopsy punch (diameter, 2 mm) through the center of the gluteus maximus (GM) in adult mice. (biomedcentral.com)
  • Following punch biopsy of adult skeletal muscle, regenerating microvessels span the wound and become perfused with blood prior to myofiber regeneration. (biomedcentral.com)
  • These properties will act mediating the action of cell adhesion proteins, regulating cell behavior and causing tissue regeneration. (bvsalud.org)
  • Isoform Alpha-7X2B and isoform Alpha-7X1B promote myoblast migration on laminin 1 and laminin 2/4, but isoform Alpha-7X1B is less active on laminin 1 (In vitro). (cusabio.com)
  • 1. Slowly thaw recombinant laminins at +4°C before use. (biolamina.com)
  • Cell therapy with young bone marrow from green fluorescent protein (GFP) transgenic mice exhibited GFP+ myofibres in aged muscle tissues. (nagoya-u.ac.jp)
  • Whilst vector administration in these studies was conducted with pharmacological interventions or in neonatal animals, rAAV8 vectors appear more efficient at crossing the blood vessel barrier and transducing heart and skeletal muscle of adult mice and hamsters (Wang et al. (justia.com)
  • Dystrophin protein is integral to the structural stability of the myofiber. (medscape.com)
  • Using a series of fusion proteins corresponding to the different structural domains of TSP, binding of [125I]DCN to TSP was found to be dependent of the N-terminal domain and, to a lesser extent, of the type 1 repeats and the C-terminal domain of TSP. (embl.de)
  • A heterogeneous family of water-soluble structural proteins found in cells of the vertebrate lens. (lookformedical.com)
  • Matrix metalloproteases (MMPs) comprise a family of enzymes that cleave protein substrates based on a conserved mechanism involving activation of an active site-bound water molecule by a Zn 2+ ion. (evelinvahter.com)
  • 1993 ). Catalysis of protein substrates is (most probably) carried out via a general base mechanism involving activation of a zinc-bound water molecule by the carboxylate group of the conserved glutamate residue in the catalytic pocket followed by attack of water on the polarized carbonyl group in the substrate's scissile bond (Browner et al. (evelinvahter.com)
  • These findings prove that alpha 7 integrin expression confers a gain of function-motile phenotype to immobile cells and may be responsible for transduction of the laminin-induced cell motility. (uea.ac.uk)
  • Widespread transduction of cardiac and skeletal muscle has been achieved in adult mouse by intravenous administration of rAAV6 vector supplemented with vascular endothelium growth factor (VEGF) (Gregorevic et al. (justia.com)
  • Biolaminin 521 (laminin-521 protein) is a key cell adhesion protein of the natural stem cell niche, expressed and secreted by human pluripotent stem cells (hPSCs) in the inner cell mass of the embryo. (biolamina.com)
  • The laminin 521 protein is also one of the most common laminins expressed in the human adult body and therefore Biolaminin 521 also supports the culture of specialized tissue cell types, such as pancreatic cells, liver cells, vascular cells, cardiac and skeletal muscle cells, and different neural cells. (biolamina.com)
  • Previously we have shown that the locomotion of murine skeletal myoblasts is specifically and significantly enhanced on laminin but not on other matrix proteins. (uea.ac.uk)
  • One of the major laminin receptors of myoblasts is the alpha 7 beta 1 integrin, which was first described in human MeWo melanoma cells and Rugli glioblastoma cells. (uea.ac.uk)
  • However, mRNA and protein levels for CD2AP and podocin were greatly reduced, suggesting a cooperative role for these molecules in foot process and slit diaphragm formation. (nih.gov)
  • Northern blotting of the transfected cells showed that the alpha 7 mRNA was expressed efficiently, and the protein was detected on the cell surface by immunofluorescence and fluorescence-activated cell sorter analysis. (uea.ac.uk)
  • The chelating binding mode accounts for the high affinity of this protein-carbohydrate interaction. (nature.com)
  • The 1,384-amino acid protein, also designated p190 or CASPR for 'contactin-associated protein,' includes an extracellular domain with several putative protein-protein interaction domains, a putative transmembrane domain, and a 74-amino acid cytoplasmic domain. (nih.gov)
  • It is well known that the adsorption of proteins onto biomaterial surfaces modulates the cellular interaction with these surfaces. (wikigenes.org)
  • The article Skeletal Muscle - Structure and Histology provides a review of normal skeletal muscle histology and ultrastructure, including the histologic appearance of normal muscle with some of the various stains that are used for the processing of muscle biopsies. (medscape.com)
  • Along with other BMPs, including BMP-2, -4 and -7, which are well known as potent osteoinductive growth factors, GDF-5 plays important roles in the development of bones, cartilage and tendons, as evidenced by the tendency for the gene and protein expression levels of GDF-5 to increase over time in these tissues. (ispub.com)
  • They are particularly active in tissues and organs in which the lifespan of the differentiated cells is short, like blood, skin and the inner cell layer covering the intestinal cavity, as well as in the repair of skeletal muscle after exercise of injury. (pas.va)
  • FKRP is one of the multiple proteins involved in the aDG glycosylation process, acting with its partner Fukutin as a ribitol-transferase. (mdaconference.org)
  • Defects of glycosylation of aDG and of its binding to laminin were observed, as well as progressive histological dystrophic signs as centronucleation, inflammation, necrosis and fibrosis. (mdaconference.org)
  • Although normally a cytoplasmic protein, plectin is expressed on the cell membrane in pancreatic ductal adenocarcinoma (PDAC) and can therefore be used to target PDAC cells. (wikipedia.org)
  • Dystrophin accounts for only a small amount of the proteins in striated muscle, but it has a vital role in the maintenance of the muscle's membrane integrity. (ayurvedapc.blog)
  • An interleukin-1 subtype that is synthesized as an inactive membrane-bound pro-protein. (lookformedical.com)
  • An 11-kDa protein associated with the outer membrane of many cells including lymphocytes. (lookformedical.com)
  • Their unique hourglass-shaped architecture is highly conserved among different bacterial membrane proteins and other biological channels. (ibecbarcelona.eu)
  • Laminins are found in an intricate lattice of proteins and other molecules that forms in the spaces between cells (the extracellular matrix). (medlineplus.gov)
  • Mock-transfected cells did not migrate significantly of alpha 7-transfected 293 cells through laminin-coated filters in a Boyden chamber assay was significantly enhanced in comparison to mock transfected cells. (uea.ac.uk)
  • Swiss 3T3 cells were grown to 25% confluency on poly-lysine and laminin coverslips. (cytoskeleton.com)
  • Our results indicate that reduced levels of proteins associated with foot processes and the glomerular slit diaphragm likely contribute, along with reduced levels of GBM collagens, to the nephropathy associated with NPS. (nih.gov)
  • Drosophila proteins Slit, Crumbs, Fat. (embl.de)
  • These underlie therefore from cases( clinical) but are typically from afferents, chamber, mechanism, and chest protein. (siriuspixels.com)
  • This new mouse model, named HSA-FKRPdel, has a normal life span but present a severe dystrophic process in the skeletal muscle. (mdaconference.org)
  • Depending on the intracellular arrangement of these myofilaments , muscle tissue is classified as either striated (skeletal and cardiac) or nonstriated (smooth) muscle. (amboss.com)
  • Laminin 221 is important for muscle development and function and is together with laminin 211 one of the main laminin isoforms present in adult muscle tissue, including varying amounts of laminin 521 and laminin 421 depending on the tissue. (biolamina.com)
  • 3. Add the diluted laminin solution to tissue culture-treated cultureware for a final coating concentration of 0.5-2 μg/cm2. (biolamina.com)
  • In muscle, plectin binds to the periphery of Z-discs, and along with the intermediate filament protein desmin, may form lateral linkages among neighboring Z-discs. (wikipedia.org)
  • Laminin G domains can vary in their function, and a variety of binding functions have been ascribed to different LamG modules. (embl.de)
  • May participate in the genesis and function of cardiac and skeletal muscle. (xenbase.org)
  • For a review of α2-laminin in skeletal muscle function, see Holmberg and Durbeej, 2012. (biolamina.com)
  • I use a range of molecular biology techniques to determine the cellular function of proteins involved in the aforementioned disorders. (cardiff.ac.uk)
  • As with most protein families, deciphering the function(s) of MMPs is difficult, as they can modify many proteins. (evelinvahter.com)