Costameres: STRIATED MUSCLE cell components which anchor the MYOFIBRILS from the Z-bands to the SARCOLEMMA and EXTRACELLULAR MATRIX. Costameric proteins include the proteins of FOCAL ADHESIONS.Vinculin: A cytoskeletal protein associated with cell-cell and cell-matrix interactions. The amino acid sequence of human vinculin has been determined. The protein consists of 1066 amino acid residues and its gene has been assigned to chromosome 10.Sarcolemma: The excitable plasma membrane of a muscle cell. (Glick, Glossary of Biochemistry and Molecular Biology, 1990)Desmin: An intermediate filament protein found predominantly in smooth, skeletal, and cardiac muscle cells. Localized at the Z line. MW 50,000 to 55,000 is species dependent.Dystrophin: A muscle protein localized in surface membranes which is the product of the Duchenne/Becker muscular dystrophy gene. Individuals with Duchenne muscular dystrophy usually lack dystrophin completely while those with Becker muscular dystrophy have dystrophin of an altered size. It shares features with other cytoskeletal proteins such as SPECTRIN and alpha-actinin but the precise function of dystrophin is not clear. One possible role might be to preserve the integrity and alignment of the plasma membrane to the myofibrils during muscle contraction and relaxation. MW 400 kDa.Myofibrils: The long cylindrical contractile organelles of STRIATED MUSCLE cells composed of ACTIN FILAMENTS; MYOSIN filaments; and other proteins organized in arrays of repeating units called SARCOMERES .Intermediate Filaments: Cytoplasmic filaments intermediate in diameter (about 10 nanometers) between the microfilaments and the microtubules. They may be composed of any of a number of different proteins and form a ring around the cell nucleus.Muscle Proteins: The protein constituents of muscle, the major ones being ACTINS and MYOSINS. More than a dozen accessory proteins exist including TROPONIN; TROPOMYOSIN; and DYSTROPHIN.Muscle, Skeletal: A subtype of striated muscle, attached by TENDONS to the SKELETON. Skeletal muscles are innervated and their movement can be consciously controlled. They are also called voluntary muscles.Cytoskeleton: The network of filaments, tubules, and interconnecting filamentous bridges which give shape, structure, and organization to the cytoplasm.Fluorescent Antibody Technique: Test for tissue antigen using either a direct method, by conjugation of antibody with fluorescent dye (FLUORESCENT ANTIBODY TECHNIQUE, DIRECT) or an indirect method, by formation of antigen-antibody complex which is then labeled with fluorescein-conjugated anti-immunoglobulin antibody (FLUORESCENT ANTIBODY TECHNIQUE, INDIRECT). The tissue is then examined by fluorescence microscopy.Actins: Filamentous proteins that are the main constituent of the thin filaments of muscle fibers. The filaments (known also as filamentous or F-actin) can be dissociated into their globular subunits; each subunit is composed of a single polypeptide 375 amino acids long. This is known as globular or G-actin. In conjunction with MYOSINS, actin is responsible for the contraction and relaxation of muscle.Myocardium: The muscle tissue of the HEART. It is composed of striated, involuntary muscle cells (MYOCYTES, CARDIAC) connected to form the contractile pump to generate blood flow.Dystrophin-Associated Protein Complex: A macromolecular complex of proteins that includes DYSTROPHIN and DYSTROPHIN-ASSOCIATED PROTEINS. It plays a structural role in the linking the CYTOSKELETON to the EXTRACELLULAR MATRIX.Dystrophin-Associated Proteins: A group of proteins that associate with DYSTROPHIN at the CELL MEMBRANE to form the DYSTROPHIN-ASSOCIATED PROTEIN COMPLEX.Dystroglycans: Dystrophin-associated proteins that play role in the formation of a transmembrane link between laminin-2 and DYSTROPHIN. Both the alpha and the beta subtypes of dystroglycan originate via POST-TRANSLATIONAL PROTEIN PROCESSING of a single precursor protein.Utrophin: An autosomally-encoded 376-kDa cytoskeletal protein that is similar in structure and function to DYSTROPHIN. It is a ubiquitously-expressed protein that plays a role in anchoring the CYTOSKELETON to the PLASMA MEMBRANE.Muscular Diseases: Acquired, familial, and congenital disorders of SKELETAL MUSCLE and SMOOTH MUSCLE.Talin: A 235-kDa cytoplasmic protein that is also found in platelets. It has been localized to regions of cell-substrate adhesion. It binds to INTEGRINS; VINCULIN; and ACTINS and appears to participate in generating a transmembrane connection between the extracellular matrix and the cytoskeleton.Tendons: Fibrous bands or cords of CONNECTIVE TISSUE at the ends of SKELETAL MUSCLE FIBERS that serve to attach the MUSCLES to bones and other structures.Muscles: Contractile tissue that produces movement in animals.Aging: The gradual irreversible changes in structure and function of an organism that occur as a result of the passage of time.Chloride-Bicarbonate Antiporters: Electroneutral chloride bicarbonate exchangers that allow the exchange of BICARBONATE IONS exchange for CHLORIDE IONS across the cellular membrane. The action of specific antiporters in this class serve important functions such as allowing the efficient exchange of bicarbonate across red blood cell membranes as they passage through capillaries and the reabsorption of bicarbonate ions by the kidney.Cardiomyopathy, Hypertrophic: A form of CARDIAC MUSCLE disease, characterized by left and/or right ventricular hypertrophy (HYPERTROPHY, LEFT VENTRICULAR; HYPERTROPHY, RIGHT VENTRICULAR), frequent asymmetrical involvement of the HEART SEPTUM, and normal or reduced left ventricular volume. Risk factors include HYPERTENSION; AORTIC STENOSIS; and gene MUTATION; (FAMILIAL HYPERTROPHIC CARDIOMYOPATHY).Sodium-Hydrogen Antiporter: A plasma membrane exchange glycoprotein transporter that functions in intracellular pH regulation, cell volume regulation, and cellular response to many different hormones and mitogens.Sodium-Calcium Exchanger: An electrogenic ion exchange protein that maintains a steady level of calcium by removing an amount of calcium equal to that which enters the cells. It is widely distributed in most excitable membranes, including the brain and heart.Antiporters: Membrane transporters that co-transport two or more dissimilar molecules in the opposite direction across a membrane. Usually the transport of one ion or molecule is against its electrochemical gradient and is "powered" by the movement of another ion or molecule with its electrochemical gradient.Bicarbonates: Inorganic salts that contain the -HCO3 radical. They are an important factor in determining the pH of the blood and the concentration of bicarbonate ions is regulated by the kidney. Levels in the blood are an index of the alkali reserve or buffering capacity.Myocardial Contraction: Contractile activity of the MYOCARDIUM.Rodenticides: Substances used to destroy or inhibit the action of rats, mice, or other rodents.Rodent Control: The reduction or regulation of the population of noxious, destructive, or dangerous rodents through chemical, biological, or other means.Cell Membrane: The lipid- and protein-containing, selectively permeable membrane that surrounds the cytoplasm in prokaryotic and eukaryotic cells.Rodent Diseases: Diseases of rodents of the order RODENTIA. This term includes diseases of Sciuridae (squirrels), Geomyidae (gophers), Heteromyidae (pouched mice), Castoridae (beavers), Cricetidae (rats and mice), Muridae (Old World rats and mice), Erethizontidae (porcupines), and Caviidae (guinea pigs).Ankyrin Repeat: Protein motif that contains a 33-amino acid long sequence that often occurs in tandem arrays. This repeating sequence of 33-amino acids was discovered in ANKYRIN where it is involved in interaction with the anion exchanger (ANION EXCHANGE PROTEIN 1, ERYTHROCYTE). Ankyrin repeats cooperatively fold into domains that mediate molecular recognition via protein-protein interactions.4-Hydroxycoumarins: Substances found in many plants, containing the 4-hydroxycoumarin radical. They interfere with vitamin K and the blood clotting mechanism, are tightly protein-bound, inhibit mitochondrial and microsomal enzymes, and are used as oral anticoagulants.Peer Group: Group composed of associates of same species, approximately the same age, and usually of similar rank or social status.Video Games: A form of interactive entertainment in which the player controls electronically generated images that appear on a video display screen. This includes video games played in the home on special machines or home computers, and those played in arcades.Videotape Recording: Recording of visual and sometimes sound signals on magnetic tape.Periodicals as Topic: A publication issued at stated, more or less regular, intervals.Journal Impact Factor: A quantitative measure of the frequency on average with which articles in a journal have been cited in a given period of time.Peer Review, Research: The evaluation by experts of the quality and pertinence of research or research proposals of other experts in the same field. Peer review is used by editors in deciding which submissions warrant publication, by granting agencies to determine which proposals should be funded, and by academic institutions in tenure decisions.Peer Review: An organized procedure carried out by a select committee of professionals in evaluating the performance of other professionals in meeting the standards of their specialty. Review by peers is used by editors in the evaluation of articles and other papers submitted for publication. Peer review is used also in the evaluation of grant applications. It is applied also in evaluating the quality of health care provided to patients.Dyneins: A family of multisubunit cytoskeletal motor proteins that use the energy of ATP hydrolysis to power a variety of cellular functions. Dyneins fall into two major classes based upon structural and functional criteria.Microtubule-Associated Proteins: High molecular weight proteins found in the MICROTUBULES of the cytoskeletal system. Under certain conditions they are required for TUBULIN assembly into the microtubules and stabilize the assembled microtubules.Tropomodulin: An actin capping protein that binds to the pointed-end of ACTIN. It functions in the presence of TROPOMYOSIN to inhibit microfilament elongation.Muscle, Striated: One of two types of muscle in the body, characterized by the array of bands observed under microscope. Striated muscles can be divided into two subtypes: the CARDIAC MUSCLE and the SKELETAL MUSCLE.Microtubules: Slender, cylindrical filaments found in the cytoskeleton of plant and animal cells. They are composed of the protein TUBULIN and are influenced by TUBULIN MODULATORS.Microfilament Proteins: Monomeric subunits of primarily globular ACTIN and found in the cytoplasmic matrix of almost all cells. They are often associated with microtubules and may play a role in cytoskeletal function and/or mediate movement of the cell or the organelles within the cell.Enediynes: Compounds with triple bonds to each side of a double bond. Many of these are CYTOTOXINS and are researched for use as CYTOTOXIC ANTIBIOTICS.Cellular Phone: Analog or digital communications device in which the user has a wireless connection from a telephone to a nearby transmitter. It is termed cellular because the service area is divided into multiple "cells." As the user moves from one cell area to another, the call is transferred to the local transmitter.Polyketide Synthases: Large enzyme complexes composed of a number of component enzymes that are found in STREPTOMYCES which biosynthesize MACROLIDES and other polyketides.Muscle Fibers, Skeletal: Large, multinucleate single cells, either cylindrical or prismatic in shape, that form the basic unit of SKELETAL MUSCLE. They consist of MYOFIBRILS enclosed within and attached to the SARCOLEMMA. They are derived from the fusion of skeletal myoblasts (MYOBLASTS, SKELETAL) into a syncytium, followed by differentiation.Mobile Applications: Computer programs or software installed on mobile electronic devices which support a wide range of functions and uses which include television, telephone, video, music, word processing, and Internet service.

An ankyrin-based mechanism for functional organization of dystrophin and dystroglycan. (1/10)

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Facioscapulohumeral muscular dystrophy region gene-1 (FRG-1) is an actin-bundling protein associated with muscle-attachment sites. (2/10)

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Ankyrin-B interactions with spectrin and dynactin-4 are required for dystrophin-based protection of skeletal muscle from exercise injury. (3/10)

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Desmoplakin and talin2 are novel mRNA targets of fragile X-related protein-1 in cardiac muscle. (4/10)

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The Mef2A transcription factor coordinately regulates a costamere gene program in cardiac muscle. (5/10)

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Phosphoinositide 3-kinase (PI3K(p110alpha)) directly regulates key components of the Z-disc and cardiac structure. (6/10)

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Cardiac-specific deletion of the microtubule-binding protein CENP-F causes dilated cardiomyopathy. (7/10)

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Substrate stiffness affects sarcomere and costamere structure and electrophysiological function of isolated adult cardiomyocytes. (8/10)

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The costamere or dystrophin-associated protein complex (DAPC) is a structural-functional component of skeletal muscle cells which, according to original descriptions in the early 1980s (which are generally still accepted), are sub-sarcolemmal protein assemblies circumferentially aligned in register with the Z-disk of peripheral myofibrils. They physically couple force-generating sarcomeres with the sarcolemma in striated muscle cells and are thus considered the "Achilles heel", i.e. the key vulnerable point of the muscle which is defective in many myopathies. [1] The DAPC contains various macromolecules such as dystroglycans which are responsible for linking the DAPC to extracellular proteins; collagen and laminin for example. Therefore it is one of the features of the sarcolemma which helps to couple the sarcomere to the extracellular connective tissue. Desmin protein may also bind to the DAPC, and regions of it are known to be involved in signaling. ...
A band, basolateral plasma membrane, costamere, cytoplasm, cytoskeleton, integral component of plasma membrane, intercalated disc, intracellular, M band, membrane raft
Ankyrin-B is a member of the ankyrin family of proteins. ankyrin-1 has shown to be essential in normal function of erythrocytes;[10] however, ankyrin-B and ankyrin-3 play essential roles in the localization and membrane stabilization of ion transporters and ion channels in cardiomyocytes.[9][11] Functional insights into ankyrin-B function have come from studies employing ankyrin-B chimeric proteins. One study showed that the death/C-terminal domain of ankyrin-B determines both the subcellular localization as well as activity in restoring normal inositol trisphosphate receptor and ryanodine receptor localization and cardiomyocyte contractility.[8] Further studies have shown that the beta-hairpin loops within the ankyrin repeat domain of ankyrin-B are required for the interaction with the inositol trisphosphate receptor, and a reduction of ankyrin-B in neonatal cardiomyocytes reduces the half-life of the inositol trisphosphate receptor by 3-fold and destabilizes its proper localization; all of ...
Human Usher syndrome (USH) is the most common form of combined deaf-blindness, characterized by profound congenital deafness, constant vestibular dysfunction and pre-pubertal onset of retinitis pigmentosa. The USH1G protein SANS (scaffold protein containing ankyrin repeats and SAM domain) is associated with microtubules and mediates a transport related periciliary protein network in photoreceptor cells. Here we aim to enlighten the involvement of SANS in ciliary transport of photoreceptor cells by identifying proteins associated with SANS in transport complexes. In Y2H screen of retinal cDNA library we identified the direct binding of SANS to dynactin-1 (p150Glued), a subunit of the dynactin complex and cargo linker to the cytoplasmic dynein motor. This interaction was validated in complementary interaction assays in vitro and in cell culture. In addition, we demonstrated the integration of SANS into the cytoplasmic dynein transport complex by GST-pull down of SANS with cytoplasmic dynein intermediate
As reported by Dr. Vino (and elsewhere), much-celebrated (and almost-as-often-maligned) consultant winemaker Michel Rolland was recently asked if there was an
We show here that talin 1 is crucial for the maintenance of integrin attachment sites at MTJs. Tln1HSA-CREko mice were viable and fertile, but suffered from a progressive myopathy. Whereas integrins and some of their effectors such as FAK, Ilk and vinculin still were localized to muscle attachment sites at costameres and MTJs, MTJs showed structural abnormalities. Defects in the ultrastructure of MTJs, such as decreased interdigitations of muscle and tendon and retraction of myofilaments from electron-dense plaques at the plasma membrane, indicate that in the absence of talin 1 the mechanical connection of actin filaments and integrins at the MTJ was compromised. By contrast, sarcolemmal integrity was largely maintained. Defects in skeletal muscle were prominent in 6- to 7-month-old mice, and were only occasionally noted in 1- to 2-month-old animals, suggesting that the defects were caused by mechanical failure of MTJs under duress. In agreement with this finding, isolated muscle fibers from 7 ...
Feeling HEADACHE while using Hydroxyzine? HEADACHE Causes, Patient Concerns and Latest Treatments and Hydroxyzine Reports and Side Effects.
Genetic instructions for developing limbs and digits were present in primitive fish millions of years before their descendants first crawled on to land, researchers have discovered.
Facioscapulohumeral muscular dystrophy (FSHD) affects over 25,000 people in the USA alone, making it one of the most prevalent genetic diseases. The genetic mutation underlying FSHD is usually a reduction in the copy number of a macrosatellite repeat on chromosome 4 referred to as D4Z4 (van Deutekom et al., 1993; Wijmenga et al., 1992). This repeat is GC-rich, highly methylated and normally subjected to repeat-induced silencing, which is disrupted in an allele-specific manner by contractions to 10 or fewer copies (van Overveld et al., 2003) or is disrupted on all D4Z4 repeats owing to mutation in the chromatin protein SMCHD1 (de Greef et al., 2009; Hartweck et al., 2013; Lemmers et al., 2012). When silencing at D4Z4 breaks down, an RNA transcript encoding the DUX4 protein (Gabriëls et al., 1999) is expressed. The presence of a poly(A) signal downstream of the D4Z4 repeats on chromosome 4 (chr4) (Dixit et al., 2007) leads to DUX4 expression and explains why disease is associated only with ...
Facioscapulohumeral muscular dystrophy (FSHD) isan enigmatic inherited disorder, while the disease locus for this condition was mapped some 17 years ago and the mutations associated with the disease are known, the exact identity of the FSHD gene remains elusive
Alpha II-spectrin, also known as Spectrin alpha chain, brain is a protein that in humans is encoded by the SPTAN1 gene. Alpha II-spectrin is expressed in a variety of tissues, and is highly expressed in cardiac muscle at Z-disc structures, costameres and at the sarcolemma membrane. Mutations in alpha II-spectrin have been associated with early infantile epileptic encephalopathy-5, and alpha II-spectrin may be a valuable biomarker for Guillain-Barré syndrome and infantile congenital heart disease. Alternate splicing of alpha II-spectrin has been documented and results in multiple transcript variants; specifically, cardiomyocytes have four identified alpha II-spectrin splice variants. As opposed to alpha I-spectrin that is principally found in erythrocytes, alpha II-spectrin is expressed in most tissues. In cardiac tissue, alpha II-spectrin is found in myocytes at Z-discs, costameres, and the sarcolemma membrane, and in cardiac fibroblasts along the surface of the cytoskeletal network. Alpha ...
Part 1 (dose escalation, open-label) Part 1 will consist of up to 6 cohorts (A to F) of patients and will evaluate multiple ascending dose levels of ACE-083 in either the tibialis anterior (TA) or biceps brachii (BB) muscle. Patients in each cohort will be enrolled in a 4-week screening period before beginning treatment. A Safety Review Team (SRT) will meet to review data for each cohort when at least 4 patients within a cohort have completed their Day 43 visit prior to dose escalation.. Part 2 (randomized, double-blind, placebo-controlled) Prior to the initiation of Part 2, a review of safety and efficacy data from Part 1 will be conducted to determine whether cohorts for one or both muscles will be pursued in Part 2, as well as the recommended dose level for each muscle. A total of up to 40 new patients (20 patients per muscle) may be enrolled and randomized (3:2) to receive either ACE-083 (n=12) or placebo (n=8) unilaterally or bilaterally (if both sides are affected per inclusion criteria) ...
Derepression of in skeletal muscle has emerged as a likely cause of pathology in facioscapulohumeral muscular dystrophy (FSHD). Here we report on the use of ...
Study showed that the variability in clinical severity of facioscapulohumeral muscular dystrophy in FSHD1 and FSHD2 individuals is dependent on individual differences in susceptibility to D4Z4 hypomethylation ...
Comments, concepts and statistics about Substrate stiffness governs the initiation of B cell activation by the concerted signaling of PKCβ and focal adhesion kinase.
TY - JOUR. T1 - Facioscapulohumeral muscular dystrophy region gene 1 Is a dynamic RNA-associated and actin-bundling protein. AU - Sun, Chia Yun Jessica. AU - Van Koningsbruggen, Silvana. AU - Long, Steven W.. AU - Straasheijm, Kirsten. AU - Klooster, Rinse. AU - Jones, Takako I.. AU - Bellini, Michel. AU - Levesque, Lyne. AU - Brieher, William M.. AU - Van Der Maarel, Silvère M.. AU - Jones, Peter L.. PY - 2011/8/12. Y1 - 2011/8/12. N2 - FSHD region gene 1 (FRG1) is a dynamic nuclear and cytoplasmic protein that, in skeletal muscle, shows additional localization to the sarcomere. Maintaining appropriate levels of FRG1 protein is critical for muscular and vascular development in vertebrates; however, its precise molecular function is unknown. This study investigates the molecular functions of human FRG1, along with mouse FRG1 and Xenopus frg1, using molecular, biochemical, and cellular-biological approaches, to provide further insight into its roles in vertebrate development. The nuclear ...
Facioscapulohumeral muscular dystrophy (FSHD), caused by partial deletion of the D4Z4 macrosatellite repeat on chromosome 4q, has a complex genetic and epigenetic etiology. To develop FSHD, D4Z4 contraction needs to occur on a specific genetic background. Only contractions associated with the 4qA161 haplotype cause FSHD.
Actin, gamma 1, encoded by this gene, is found in non-muscle cells in the cytoplasm, and in muscle cells at costamere ... In myocytes, sarcomeres adhere to the sarcolemma via costameres, which align at Z-discs and M-lines. The two primary ... Danowski BA, Imanaka-Yoshida K, Sanger JM, Sanger JW (Sep 1992). "Costameres are sites of force transmission to the substratum ... It has been shown that gamma-actin interacting with another costameric protein dystrophin is critical for costameres forming ...
... is expressed at focal adhesions of non-striated cells and at costameres of striated muscle cells, and it functions to ... Costameric proteins redistribute to form mature costameres. While the precise functions of paxillin in this process are still ... In striated muscle cells, paxillin is important in costamerogenesis, or the formation of costameres, which are specialized ... Premyofibrils become nascent myofibrils, which progressively align to form mature myofibrils and nascent costamere structures ...
PKCε binds RACK2 at Z-lines with an EC50 of 86 nM; PKCε also binds at costameres to syndecan-4. PKCε has been shown to bind F- ... A role for focal adhesion kinase at costameres in strain-sensing and modulation of sarcomere length has been linked to ... "Localization of the transmembrane proteoglycan syndecan-4 and its regulatory kinases in costameres of rat cardiomyocytes: a ...
In muscle, CAP/Ponsin plays a role in the formation of mature costameres from focal adhesion-like contacts during assembly of ... CAP/Ponsin is localized to costamere structures. CAP/Ponsin may exist as thirteen alternatively-spliced isoforms, ranging from ... "Paxillin and ponsin interact in nascent costameres of muscle cells". Journal of Molecular Biology. 369 (3): 665-82. doi:10.1016 ...
Wu JC, Sung HC, Chung TH, DePhilip RM (2002). "Role of N-cadherin- and integrin-based costameres in the development of rat ... Mondello MR, Bramanti P, Cutroneo G, Santoro G, Di Mauro D, Anastasi G (Jul 1996). "Immunolocalization of the costameres in ... and is localized to costamere structures in cardiac and skeletal muscle cells, and to focal adhesions in smooth muscle and non- ... talin-1 is found complexed to integrins and localized to intercalated discs of cardiac muscle and to costamere structures of ...
At the costameres, another region implicated in force transmission, MLP is thought to be contributing in mechanosensing through ... However, the precise role of MLP at the costameres has not been extensively investigated yet. At the microfilaments, MLP is ... the costameres, and the microfilaments. At the Z-line, MLP interacts with different Z-line components and acts as a scaffold ... costameres, where it binds to zyxin, integrin linked kinase (ILK) and beta1-spectrin; intercalated discs, where it associates ...
... contributes to linkage between costameres and the contractile apparatus in skeletal muscle of synemin null animals. ...
This complex is variously known as the costamere or the dystrophin-associated protein complex. Many muscle proteins, such as α- ... as shown in recent studies where biomechanical properties of the sarcolemma and its links through costameres to the contractile ... "Biomechanics of the sarcolemma and costameres in single skeletal muscle fibers from normal and dystrophin- null mice". J Muscle ... dystrobrevin, syncoilin, synemin, sarcoglycan, dystroglycan, and sarcospan, colocalize with dystrophin at the costamere. The ...
Galie, PA; Khalid, N; Carnahan, KE; Westfall, MV; Stegemann, JP (NaN). "Substrate stiffness affects sarcomere and costamere ... is localized to Z-discs and costamere structures, bound to the four and one half LIM domain proteins, FHL1 and FHL2. It has ... and localizes to Z-disc and costamere structures. Mutations in ITGA7 have been associated with congenital myopathies and ...
Knockout of FXR1 upregulates talin-2 protein, which disrupts the architecture of desmosomes and costameres in cardiac muscle. ... In mature cardiomyocytes and skeletal muscle, talin-2 is expressed at costameres and intercalated discs, thus demonstrating ... levels in cardiac muscle and functions to provide linkages between the extracellular matrix and actin cytoskeleton at costamere ...
... is a protein that binds to dystrophin in the costamere of skeletal muscle cells. There are at least two isoforms ...
Ankyrin-B has also been identified to associate at sarcomeric M-lines and costameres in cardiac muscle and skeletal muscle, ... as well as in costamere structures. Mutations in ankyrin-B cause a dominantly-inherited, cardiac arrhythmia syndrome known as ... which is required for proper localization and functioning of the dystrophin complex and costamere structures, as well as ...
"Biomechanics of the sarcolemma and costameres in single skeletal muscle fibers from normal and dystrophin- null mice". J Muscle ...
... and costameres in skeletal muscles. Metavinculin tail domain has a lower affinity for the head as compared with the vinculin ...
In cardiac tissue, alpha II-spectrin is found in myocytes at Z-discs, costameres, and the sarcolemma membrane, and in cardiac ... costameres and at the sarcolemma membrane. Mutations in alpha II-spectrin have been associated with early infantile epileptic ...
Costamere Dystrophin-Associated Protein Complex at the US National Library of Medicine Medical Subject Headings (MeSH) Ehmsen J ...
Integrin beta-1D is specifically localized to costameres and intercalated discs of cardiac muscle and costameres, myotendinous ... In cardiac muscle and skeletal muscle, the integrin beta-1D isoform is specifically expressed, and localizes to costameres, ...
The costamere is a structural-functional component of striated muscle cells which connects the sarcomere of the muscle to the ... Costameres at the US National Library of Medicine Medical Subject Headings (MeSH) Srivastava, D.; Yu, S (2006). "Stretching to ... Costameres are sub-sarcolemmal protein assemblies circumferentially aligned in register with the Z-disk of peripheral ... García-Pelagio Karla; Bloch Robert; Ortega A; Gonzáles-Serratos Hugo (2011). "Biomechanics of the sarcolemma and costameres in ...
Primary type Ia sensory fibers (large diameter) spiral around all intrafusal muscle fibres, ending near the middle of each fibre. Secondary type II sensory fibers (medium diameter) end adjacent to the central regions of the static bag and chain fibres.[2] These fibres send information by stretch-sensitive mechanically-gated ion-channels of the axons.[3] The motor part of the spindle is provided by motor neurons: up to a dozen gamma motor neurons and one or two beta motor neurons, collectively called fusimotor neurons.[citation needed] These activate the muscle fibres within the spindle. Gamma motor neurons supply only muscle fibres within the spindle, whereas beta motor neurons supply muscle fibres both within and outside of the spindle. Activation of the neurons causes a contraction and stiffening of the end parts of the muscle spindle muscle fibers. Fusimotor neurons are classified as static or dynamic according to the type of muscle fibers they innervate and their effects on the responses of ...
... s are skeletal muscle fibers that serve as specialized sensory organs (proprioceptors) that detect the amount and rate of change in length of a muscle.[1] They constitute the muscle spindle and are innervated by both sensory (afferent) and motor (efferent) fibers. Gamma efferents from small multipolar cells from anterior gray column innervate it. These form a part of neuromuscular spindles. Intrafusal muscle fibers are walled off from the rest of the muscle by an outer connective tissue sheath consisting of flattened fibroblasts and collagen.[2] This sheath has a spindle or "fusiform" shape, hence the name "intrafusal". There are two types of intrafusal muscle fibers: nuclear bag and nuclear chain fibers. They bear two types of sensory ending, known as annulospiral and flower-spray endings. Both ends of these fibers contract but the central region only stretches and does not contract. They are innervated by gamma motor neurons and beta motor neurons. It is by the sensory ...
... s are an important link in the chain from electrical excitation of a cell to its subsequent contraction (excitation-contraction coupling). When contraction of a muscle is needed, stimulation from a nerve or an adjacent muscle cell causes a characteristic flow of charged particles across the cell membrane known as an action potential. At rest, there are fewer positively charged particles on the inner side of the membrane compared to the outer side, and the membrane is described as being polarised. During an action potential, positively charged particles (predominantly sodium and calcium ions) flow across the membrane from the outside to the inside. This reverses the normal imbalance of charged particles and is referred to as depolarisation. One region of membrane depolarises adjacent regions, and the resulting wave of depolarisation then spreads along the cell membrane.[8] The polarisation of the membrane is restored as potassium ions flow back across the membrane from the inside to the ...
Individual muscles tend to be a mixture of various fiber types, but their proportions vary depending on the actions of that muscle and the species. For instance, in humans, the quadriceps muscles contain ~52% type I fibers, while the soleus is ~80% type I.[29] The orbicularis oculi muscle of the eye is only ~15% type I.[29] Motor units within the muscle, however, have minimal variation between the fibers of that unit. It is this fact that makes the size principal of motor unit recruitment viable. The total number of skeletal muscle fibers has traditionally been thought not to change. It is believed there are no sex or age differences in fiber distribution; however, proportions of fiber types vary considerably from muscle to muscle and person to person. Sedentary men and women (as well as young children) have 45% type II and 55% type I fibers.[citation needed] People at the higher end of any sport tend to demonstrate patterns of fiber distribution e.g. endurance athletes show a higher level of ...
The central nervous system is responsible for the orderly recruitment of motor neurons, beginning with the smallest motor units.[3] Henneman's size principle indicates that motor units are recruited from smallest to largest based on the size of the load. For smaller loads requiring less force, slow twitch, low-force, fatigue-resistant muscle fibers are activated prior to the recruitment of the fast twitch, high-force, less fatigue-resistant muscle fibers. Larger motor units are typically composed of faster muscle fibers that generate higher forces.[4] The central nervous system has two distinct ways of controlling the force produced by a muscle through motor unit recruitment: spatial recruitment and temporal recruitment. Spatial recruitment is the activation of more motor units to produce a greater force. Larger motor units contract along with small motor units until all muscle fibers in a single muscle are activated, thus producing the maximum muscle force. Temporal motor unit recruitment, or ...
The changes that occur to the myofilament in response to exercise have long been a subject of interest to exercise physiologists and the athletes who depend on their research for the most advanced training techniques. Athletes across a spectrum of sporting events are particularly interested to know what type of training protocol will result in maximal force generation from a muscle or set of muscles, so much attention has been given to changes in the myofilament under bouts of chronic and acute forms of exercise. While the exact mechanism of myofilament alteration in response to exercise is still being studied in mammals, some interesting clues have been revealed in Thoroughbred race horses. Researchers studied the presence of mRNA in skeletal muscle of horses at three distinct times; immediately before training, immediately after training, and four hours after training. They reported statistically significant differences in mRNA for genes specific to production of actin. This study provides ...
Early descriptions of "gap junctions" and "connexons" did not refer to them as such and many other terms were used. It is likely that "synaptic disks"[115] were an accurate reference to gap junction plaques. While the detailed structure and function of the connexon was described in a limited way at the time the gross "disk" structure was relatively large and easily seen by various TEM techniques. Disks allowed researchers using TEM to easily locate the connexons contained within the disk like patches in vivo and in vitro. The disk or "plaque" appeared to have structural properties different from those imparted by the connexons alone.[25] It was thought that if the area of membrane in the plaque transmitted signals the area of membrane would have to be sealed in some way to prevent leakage.[116] Later studies showed gap junction plaques are home to non-connexin proteins making the modern usage of the terms "gap junction" and "gap junction plaque" non-interchangeable as the area of the gap ...
The costamere or dystrophin-associated protein complex (DAPC) is a structural-functional component of skeletal muscle cells ... James M. Ervasti Costameres: the Achilles Heel of Herculean Muscle J. Biol. Chem. 278: 13591-13594. http://www.jbc.org/cgi/ ... Retrieved from "https://www.wikidoc.org/index.php?title=Costamere&oldid=672007" ...
Z-line or in costameres. PMID: 15558188. ...
The costamere is a structural-functional component of striated muscle cells which connects the sarcomere of the muscle to the ... Costameres at the US National Library of Medicine Medical Subject Headings (MeSH) Srivastava, D.; Yu, S (2006). "Stretching to ... Costameres are sub-sarcolemmal protein assemblies circumferentially aligned in register with the Z-disk of peripheral ... García-Pelagio Karla; Bloch Robert; Ortega A; Gonzáles-Serratos Hugo (2011). "Biomechanics of the sarcolemma and costameres in ...
Here we examine the role of desmin, the major intermediate filament protein of muscle in organizing costameres. In control ... Cytokeratins concentrate at the sarcolemma at all three domains of costameres when the latter are retained in desmin -/- muscle ... In tibialis anterior muscle from mice lacking desmin due to homologous recombination, most costameres are lost. In myofibers ... Our results suggest that desmin associates with and selectively stabilizes the Z line domains of costameres, but that ...
costamere TAS Traceable Author Statement. more info. PubMed cytosol TAS Traceable Author Statement. more info ...
costamere TAS Traceable Author Statement. more info. PubMed cytoplasm IDA Inferred from Direct Assay. more info ...
Trypanosoma cruzi infection disrupts vinculin costameres in cardiomyocytes.. Melo TG, Almeida DS, de Meirelles Mde N, Pereira ...
Talin 1 is not essential for costamere assembly. (A-D) Longitudinal and (E-L) cross-sections of muscle from 6-month-old wild- ... Talin 1 is not essential for the assembly of integrin complexes at costameres. Talin binds to proteins that are localized to ... In skeletal muscle fibers, talin 1 is localized to costameres and MTJs (Tidball et al., 1986), and its expression is regulated ... We show here that talin 1 is not essential for the assembly of integrin β1-dependent adhesion complexes at costameres and MTJs ...
Keywords: Slc4a3, Slc4a4, NBCe1, SERCA2, NCX1, costamere, protein kinase B. Citation: Prasad V, Lorenz JN, Lasko VM, Nieman ML ... AE3 has been localized to t-tubules and to the sarcolemma, where it appears to be associated with costameres (Alvarez et al., ... Samarel, A. M. (2005). Costameres, focal adhesions, and cardiomyocyte mechanotransduction. Am. J. Physiol. Heart Circ. Physiol ... Costameres form structural links between Z-discs and the extracellular matrix and serve as integrative nodal points in the ...
... costamere, cytoplasm, cytoskeleton, integral component of plasma membrane, intercalated disc, intracellular, M band, membrane ... costamere Source: RGDInferred from sequence orthologyi*16292983. *cytoplasm Source: RGDInferred from sequence orthologyi* ...
p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.,/p> ,p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.,/p> ,p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).,/p> ,p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x,sup>64,/sup> + x,sup>4,/sup> + x,sup>3,/sup> + x + 1. The algorithm is described in the ISO 3309 standard. ,/p> ,p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.,br /> ,strong>Cyclic redundancy and other checksums,/strong>,br /> ,a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993),/a>),/p> Checksum:i ...
costamere Source: Ensembl. *cytoplasm Source: GO_CentralInferred from biological aspect of ancestori*21873635 ...
Together with KRT8, helps to link the contractile apparatus to dystrophin at the costameres of striated muscle. ... Together with KRT8, helps to link the contractile apparatus to dystrophin at the costameres of striated muscle.1 Publication. , ... Observed in muscle fibers accumulating in the costameres of myoplasm at the sarcolemma in structures that contain dystrophin ...
costamere ISO Inferred from Sequence Orthology. more info. cytoplasm ISO Inferred from Sequence Orthology. more info ...
Wu JC, Sung HC, Chung TH, DePhilip RM (2002). "Role of N-cadherin- and integrin-based costameres in the development of rat ... Mondello MR, Bramanti P, Cutroneo G, Santoro G, Di Mauro D, Anastasi G (Jul 1996). "Immunolocalization of the costameres in ... and is localized to costamere structures in cardiac and skeletal muscle cells, and to focal adhesions in smooth muscle and non- ... talin-1 is found complexed to integrins and localized to intercalated discs of cardiac muscle and to costamere structures of ...
In obscurin KO mice, localization at costameres of dystrophin, but not of ?-dystroglycan, was altered, and the subsarcolemma ... dystroglycan at costameres. In this paper, we report that in mice deficient for obscurin, ankB was displaced from its ... localization of dystrophin at costameres, and maintenance of sarcolemmal integrity. ...
Bloch, R. J., and Gonzalez-Serratos, H. (2003). Lateral force transmission across costameres in skeletal muscle. Exerc. Sport ... located in the costamere region of skeletal muscle fibers and is sensitive to changes in mechanical loading (Bloch and Gonzalez ... costameres, and cytoskeleton in human soleus and vastus lateralis muscles after a long-term bedrest with or without exercise. ...
... this interaction is required for localization at costameres.By similarity1 Publication. ,p>Manually curated information for ... costamere localization requires the presence of ANK2.By similarity ...
Co-localises with the DGC at the costamere. composed of integrin, talin and viculin. force transducer. ... 1 and If - localised to costameres. 1d - associated with the z-disc. 1b - localised to mitochondria ... What are the protein complexes that transmit the force at the costamere ... links adjacent myofibres at the level of the Z disc and binds myofibrils to the sarcolemma at the level of the costameres ...
Monoclonal Antibody Costamere Monoclonal Antibody Costamere: Monoclonal Antibody - Pan-Keratin (C11) Mouse mAb, UniProt ID ...
Costamere Inc. (CMRE) Costamere leases out container ships to several major shipping companies. Like Safe Bulkers, they diod ... Costamere is up 33.99 percent on the year to hit $17.74 a share. ...
Costamere. The structural-functional component of striated myofibres that links the sarcomere to the cell membrane. ... Dystrophin is enriched at the costameres and myotendinous junctions (MTJs; Box 1) where force is transmitted across the cell ...
Actin, gamma 1, encoded by this gene, is found in non-muscle cells in the cytoplasm, and in muscle cells at costamere ... In myocytes, sarcomeres adhere to the sarcolemma via costameres, which align at Z-discs and M-lines. The two primary ... Danowski BA, Imanaka-Yoshida K, Sanger JM, Sanger JW (Sep 1992). "Costameres are sites of force transmission to the substratum ... It has been shown that gamma-actin interacting with another costameric protein dystrophin is critical for costameres forming ...
Paxillin is expressed at focal adhesions of non-striated cells and at costameres of striated muscle cells, and it functions to ... Costameric proteins redistribute to form mature costameres. While the precise functions of paxillin in this process are still ... In striated muscle cells, paxillin is important in costamerogenesis, or the formation of costameres, which are specialized ... Premyofibrils become nascent myofibrils, which progressively align to form mature myofibrils and nascent costamere structures ...
  • Finally, costameres have received considerable attention because one of their core constituents, dystrophin, is the product of the gene mutated in Duchenne muscular dystrophy ( Williams and Bloch 1999 ). (rupress.org)
  • This diagram is provided to orient the reader to the location of vinculin and talin (arrows) within the cell, but is clearly a simplified version of costamere and ICD. (nih.gov)
  • The dominant features here are costameres: discrete, rectilinear domains arrayed in transverse stripes and aligned with the Z-lines of the sarcomeres ( Berthier and Blaineau 1997 ). (rupress.org)
  • Tension is signaled through the integrin adhesion complexes found at muscle insertions and costameres but the relative importance of signalling during cardiomyocyte growth versus remodelling has not been assessed. (sdbonline.org)
  • Fak activation by mechanical stress was accompanied by a reduction of Fak/myosin heavy chain association and its relocation at subcellular sites such as costameres, Z-discs, and nuclei. (ahajournals.org)
  • At the costameres, another region implicated in force transmission, MLP is thought to be contributing in mechanosensing through its interactions with β1 spectrin and zyxin. (wikipedia.org)
  • Costameres, dense plaques and podosomes: the cell matrix adhesions in cardiovascular mechanosensing. (nih.gov)