Sarcolemma: The excitable plasma membrane of a muscle cell. (Glick, Glossary of Biochemistry and Molecular Biology, 1990)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.Muscular Dystrophy, AnimalUtrophin: 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.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 Proteins: A group of proteins that associate with DYSTROPHIN at the CELL MEMBRANE to form the DYSTROPHIN-ASSOCIATED PROTEIN COMPLEX.Mice, Inbred mdx: A strain of mice arising from a spontaneous MUTATION (mdx) in inbred C57BL mice. This mutation is X chromosome-linked and produces viable homozygous animals that lack the muscle protein DYSTROPHIN, have high serum levels of muscle ENZYMES, and possess histological lesions similar to human MUSCULAR DYSTROPHY. The histological features, linkage, and map position of mdx make these mice a worthy animal model of DUCHENNE MUSCULAR DYSTROPHY.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.Caveolin 3: A caveolin that is expressed exclusively in MUSCLE CELLS and is sufficient to form CAVEOLAE in SARCOLEMMA. Mutations in caveolin 3 are associated with multiple muscle diseases including DISTAL MYOPATHY and LIMB-GIRDLE MUSCULAR DYSTROPHY.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.Sarcoglycans: A family of transmembrane dystrophin-associated proteins that play a role in the membrane association of the DYSTROPHIN-ASSOCIATED PROTEIN COMPLEX.Muscles: Contractile tissue that produces movement in animals.Muscular Dystrophies: A heterogeneous group of inherited MYOPATHIES, characterized by wasting and weakness of the SKELETAL MUSCLE. They are categorized by the sites of MUSCLE WEAKNESS; AGE OF ONSET; and INHERITANCE PATTERNS.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 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.4-Nitrophenylphosphatase: An enzyme that catalyzes the hydrolysis of nitrophenyl phosphates to nitrophenols. At acid pH it is probably ACID PHOSPHATASE (EC 3.1.3.2); at alkaline pH it is probably ALKALINE PHOSPHATASE (EC 3.1.3.1). EC 3.1.3.41.Sodium-Potassium-Exchanging ATPase: An enzyme that catalyzes the active transport system of sodium and potassium ions across the cell wall. Sodium and potassium ions are closely coupled with membrane ATPase which undergoes phosphorylation and dephosphorylation, thereby providing energy for transport of these ions against concentration gradients.Muscular Dystrophy, Duchenne: An X-linked recessive muscle disease caused by an inability to synthesize DYSTROPHIN, which is involved with maintaining the integrity of the sarcolemma. Muscle fibers undergo a process that features degeneration and regeneration. Clinical manifestations include proximal weakness in the first few years of life, pseudohypertrophy, cardiomyopathy (see MYOCARDIAL DISEASES), and an increased incidence of impaired mentation. Becker muscular dystrophy is a closely related condition featuring a later onset of disease (usually adolescence) and a slowly progressive course. (Adams et al., Principles of Neurology, 6th ed, p1415)Calcium: A basic element found in nearly all organized tissues. It is a member of the alkaline earth family of metals with the atomic symbol Ca, atomic number 20, and atomic weight 40. Calcium is the most abundant mineral in the body and combines with phosphorus to form calcium phosphate in the bones and teeth. It is essential for the normal functioning of nerves and muscles and plays a role in blood coagulation (as factor IV) and in many enzymatic processes.Nitric Oxide Synthase Type I: A CALCIUM-dependent, constitutively-expressed form of nitric oxide synthase found primarily in NERVE TISSUE.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.Sarcoplasmic Reticulum: A network of tubules and sacs in the cytoplasm of SKELETAL MUSCLE FIBERS that assist with muscle contraction and relaxation by releasing and storing calcium ions.Freeze Fracturing: Preparation for electron microscopy of minute replicas of exposed surfaces of the cell which have been ruptured in the frozen state. The specimen is frozen, then cleaved under high vacuum at the same temperature. The exposed surface is shadowed with carbon and platinum and coated with carbon to obtain a carbon replica.Shadowing (Histology): The technique of spraying a tissue specimen with a thin coat of a heavy metal such as platinum. The specimen is sprayed from an oblique angle, which results in the uneven deposition of the coating. The varying thicknesses create a shadow effect and give a three-dimensional appearance to the specimen.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.Caveolins: The main structural proteins of CAVEOLAE. Several distinct genes for caveolins have been identified.Wheat Germ Agglutinins: Lectins purified from the germinating seeds of common wheat (Triticum vulgare); these bind to certain carbohydrate moieties on cell surface glycoproteins and are used to identify certain cell populations and inhibit or promote some immunological or physiological activities. There are at least two isoforms of this lectin.Sodium: A member of the alkali group of metals. It has the atomic symbol Na, atomic number 11, and atomic weight 23.Microscopy, Electron: Microscopy using an electron beam, instead of light, to visualize the sample, thereby allowing much greater magnification. The interactions of ELECTRONS with specimens are used to provide information about the fine structure of that specimen. In TRANSMISSION ELECTRON MICROSCOPY the reactions of the electrons that are transmitted through the specimen are imaged. In SCANNING ELECTRON MICROSCOPY an electron beam falls at a non-normal angle on the specimen and the image is derived from the reactions occurring above the plane of the specimen.Plectin: A cytoskeletal linker protein with a molecular weight of greater than 500 kDa. It binds INTERMEDIATE FILAMENTS; MICROTUBULES; and ACTIN CYTOSKELETON and plays a central role in the organization and stability of the CYTOSKELETON. Plectin is phosphorylated by CALMODULIN KINASE; PROTEIN KINASE A; and PROTEIN KINASE C.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 .Myocytes, Cardiac: Striated muscle cells found in the heart. They are derived from cardiac myoblasts (MYOBLASTS, CARDIAC).Dogs: The domestic dog, Canis familiaris, comprising about 400 breeds, of the carnivore family CANIDAE. They are worldwide in distribution and live in association with people. (Walker's Mammals of the World, 5th ed, p1065)Heart Ventricles: The lower right and left chambers of the heart. The right ventricle pumps venous BLOOD into the LUNGS and the left ventricle pumps oxygenated blood into the systemic arterial circulation.Cytoskeletal Proteins: Major constituent of the cytoskeleton found in the cytoplasm of eukaryotic cells. They form a flexible framework for the cell, provide attachment points for organelles and formed bodies, and make communication between parts of the cell possible.Aquaporin 4: Aquaporin 4 is the major water-selective channel in the CENTRAL NERVOUS SYSTEM of mammals.Ligusticum: A plant genus of the family APIACEAE.Chemistry, Bioinorganic: Field of chemistry pertaining to the study of inorganic compounds or ions and their interactions with biological ligands at the molecular level.Calcium-Transporting ATPases: Cation-transporting proteins that utilize the energy of ATP hydrolysis for the transport of CALCIUM. They differ from CALCIUM CHANNELS which allow calcium to pass through a membrane without the use of energy.Rabbits: The species Oryctolagus cuniculus, in the family Leporidae, order LAGOMORPHA. Rabbits are born in burrows, furless, and with eyes and ears closed. In contrast with HARES, rabbits have 22 chromosome pairs.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.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.Membrane Proteins: Proteins which are found in membranes including cellular and intracellular membranes. They consist of two types, peripheral and integral proteins. They include most membrane-associated enzymes, antigenic proteins, transport proteins, and drug, hormone, and lectin receptors.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.Heart: The hollow, muscular organ that maintains the circulation of the blood.Myocardial Contraction: Contractile activity of the MYOCARDIUM.Muscle Contraction: A process leading to shortening and/or development of tension in muscle tissue. Muscle contraction occurs by a sliding filament mechanism whereby actin filaments slide inward among the myosin filaments.Glucose Transporter Type 4: A glucose transport protein found in mature MUSCLE CELLS and ADIPOCYTES. It promotes transport of glucose from the BLOOD into target TISSUES. The inactive form of the protein is localized in CYTOPLASMIC VESICLES. In response to INSULIN, it is translocated to the PLASMA MEMBRANE where it facilitates glucose uptake.Cell Fractionation: Techniques to partition various components of the cell into SUBCELLULAR FRACTIONS.Muscular Diseases: Acquired, familial, and congenital disorders of SKELETAL MUSCLE and SMOOTH MUSCLE.Muscle Fibers, Fast-Twitch: Skeletal muscle fibers characterized by their expression of the Type II MYOSIN HEAVY CHAIN isoforms which have high ATPase activity and effect several other functional properties - shortening velocity, power output, rate of tension redevelopment. Several fast types have been identified.Receptors, Albumin: Cell surface proteins that bind albumin with high affinity and trigger intracellular changes influencing the behavior of cells.Neuromuscular Junction: The synapse between a neuron and a muscle.Saxitoxin: A compound that contains a reduced purine ring system but is not biosynthetically related to the purine alkaloids. It is a poison found in certain edible mollusks at certain times; elaborated by GONYAULAX and consumed by mollusks, fishes, etc. without ill effects. It is neurotoxic and causes RESPIRATORY PARALYSIS and other effects in MAMMALS, known as paralytic SHELLFISH poisoning.Spectrin: A high molecular weight (220-250 kDa) water-soluble protein which can be extracted from erythrocyte ghosts in low ionic strength buffers. The protein contains no lipids or carbohydrates, is the predominant species of peripheral erythrocyte membrane proteins, and exists as a fibrous coating on the inner, cytoplasmic surface of the membrane.Ouabain: A cardioactive glycoside consisting of rhamnose and ouabagenin, obtained from the seeds of Strophanthus gratus and other plants of the Apocynaceae; used like DIGITALIS. It is commonly used in cell biological studies as an inhibitor of the NA(+)-K(+)-EXCHANGING ATPASE.Dihydroalprenolol: Hydrogenated alprenolol derivative where the extra hydrogens are often tritiated. This radiolabeled form of ALPRENOLOL, a beta-adrenergic blocker, is used to label the beta-adrenergic receptor for isolation and study.Myotonia: Prolonged failure of muscle relaxation after contraction. This may occur after voluntary contractions, muscle percussion, or electrical stimulation of the muscle. Myotonia is a characteristic feature of MYOTONIC DISORDERS.Cell Membrane: The lipid- and protein-containing, selectively permeable membrane that surrounds the cytoplasm in prokaryotic and eukaryotic cells.Nitrendipine: A calcium channel blocker with marked vasodilator action. It is an effective antihypertensive agent and differs from other calcium channel blockers in that it does not reduce glomerular filtration rate and is mildly natriuretic, rather than sodium retentive.Annexin A6: Protein of the annexin family with a probable role in exocytotic and endocytotic membrane events.Ca(2+) Mg(2+)-ATPaseCalcium-Binding Proteins: Proteins to which calcium ions are bound. They can act as transport proteins, regulator proteins, or activator proteins. They typically contain EF HAND MOTIFS.Subcellular Fractions: Components of a cell produced by various separation techniques which, though they disrupt the delicate anatomy of a cell, preserve the structure and physiology of its functioning constituents for biochemical and ultrastructural analysis. (From Alberts et al., Molecular Biology of the Cell, 2d ed, p163)Cocos: A plant genus of the family ARECACEAE. It is a tropical palm tree that yields a large, edible hard-shelled fruit from which oil and fiber are also obtained.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.Immunohistochemistry: Histochemical localization of immunoreactive substances using labeled antibodies as reagents.
Intrafusal muscle fiber
Distrofina, a enciclopedia libre
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Basal laminaMembraneFibersSarcoplasmicExtracellular matrixContractionLocalizes to the sarcolemmaProteinsCytoplasmReceptorsActinProteinTransverse tubulesDystrophin-glycoprotein complexPenetrate deepTargeting of nNOSMyofibrilsEnclosed in a sheathNeuromuscularMyocytesCellConnective tissueMoleculesCardiac muscleMusclesIntegritySmoothMyofibersStructuralSynapticGlycoprotein complexMuscular dystrophiesMutationsLimb-Girdle MuCalciumAmino AcidSyntrophinCardiomyocytesLocalizationFunctionalMuscle tissueDifferentiateAttachmentTissue
Basal lamina8
- However, there are two other components which form the sarcolemma - basal lamina and reticular fibers. (wikipedia.org)
- These functions largely rely on myogenic stem cells, that is, satellite cells located between the sarcolemma and basal lamina. (thefreelibrary.com)
- Satellite cells are mononuclear progenitor cells found in mature muscle between the basal lamina and sarcolemma. (bionity.com)
- SCs are a population of muscle stem cells that reside between the sarcolemma and the basal lamina. (asm.org)
- Muscle cells are long, cylindrical structures that are bound by a plasma membrane (the sarcolemma) and an overlying basal lamina and when grouped into bundles (fascicles) they make up muscle. (eku.edu)
- They can be characterized as small, mononuclear cells located between the basement membrane of a muscle fiber (called the basal lamina) and the sarcolemma (the polarized plasma membrane). (ideafit.com)
- 12 The dystrophin protein is expressed at the sarcolemma of cardiac and skeletal muscle ( Figure 1 ) where it interacts with integral membrane proteins assembled in the dystrophin-glycoprotein complex forming a bridge connecting the basal lamina of the extracellular matrix to the inner cytoskeleton. (jaoa.org)
- Indeed, in the absence of any environmental stimuli, the satellite cells are in a quiescent state and are located in a specialized niche between the sarcolemma and the basal lamina 3,4 . (jove.com)
Membrane35
- Vinculin, α-actinin, β-dystroglycan and utrophin were all retained on mdx sarcolemma, indicating that the loss of costameric actin was not due to generalized membrane instability. (rupress.org)
- These action potentials travel along the cell membrane (sarcolemma), as impulses, passing from one cell to the next through channels, in structures known as gap junctions. (wikipedia.org)
- It has been shown that gamma-actin interacting with another costameric protein dystrophin is critical for costameres forming mechanically strong links between the cytoskeleton and the sarcolemmal membrane. (wikipedia.org)
- The internal compartments formed by the inner membrane of the mitochondria. (brainscape.com)
- 2. Sarcolemma: Plasma membrane of muscle fiber. (scribd.com)
- The sarcolemma was historically synonymous with the cell membrane of a striated muscle fiber or muscle cell. (wikipedia.org)
- The cell membrane of the sarcolemma receives and conducts stimuli. (wikipedia.org)
- The sarcolemma is the cell membrane enclosing each muscle fiber (muscle cell). (newworldencyclopedia.org)
- Neuromuscular junctions (NMJs) develop in a series of steps in which the postsynaptic membrane first forms by concentrating acetylcholine receptors (AChRs) and associated proteins in a flat topology. (jneurosci.org)
- The Cavin family of caveolar proteins can form membrane remodeling oligomers and thus may also impact skeletal muscle function. (pubmedcentralcanada.ca)
- Caveolae, micro-domains in the cell membrane, are known to be important in forming muscle-specific membrane structures such as the T-tubule network and play a role in some forms of muscular dystrophy. (pubmedcentralcanada.ca)
- Presynaptic motor axons are demyelinated and stop 30 nanometers from the sarcolemma , the cell membrane of a muscle cell. (primidi.com)
- The sarcolemma has invaginations called postjunctional folds , which increase the surface area of the membrane exposed to the synaptic cleft . (primidi.com)
- The outer cell membrane is called sarcolemma , and it also has smooth endoplasmic reticulum known as sarcoplasmic reticulum . (chegg.com)
- It binds to the receptors on the sarcolemma of the muscle cell causing a local change in the voltage of the membrane. (chegg.com)
- Dystrophin links actinin the cytoskeleton and dystroglycans of the muscle cell plasma membrane, known as the sarcolemma (extracellular). (boloji.com)
- The 'dystrophin-glycoprotein complex' helps anchor the structural skeleton (cytoskeleton) within the muscle cells, through the outer membrane (sarcolemma) of each cell, to the tissue framework (extracellular matrix) that surrounds each cell. (boloji.com)
- LAMA2 is known to bind directly to α-dystroglycan, the component of the DGC most distal to the sarcolemma, and thereby to anchor the muscle cell membrane to the ECM ( 16 ). (pnas.org)
- The N-terminal domain of dystrophin binds to actin, and the cysteine rich and C-terminal domains of dystrophin bind to dystrophin-glycoprotein complex (DGC), a multimeric protein complex found at the plasma membrane (sarcolemma) of muscle fibers (aka myofibers) [ 5, 6 ]. (iospress.com)
- Along with DGC, dystrophin crucially links the actin cytoskeleton of the sarcolemma to the extracellular basement membrane, as illustrated in Figure 1 [ 5, 7 ]. (iospress.com)
- Another group of cells, the myosatellite cells are found between the basement membrane and the sarcolemma of muscle fibers. (wikipedia.org)
- The cell membrane is called the sarcolemma with the cytoplasm known as the sarcoplasm . (wikipedia.org)
- Loss of localization of the muscle-related molecule nNOS at the muscle cell membrane (which is known as the sarcolemma) is also observed in DMD and has been linked to muscle damage. (medicalnewstoday.com)
- The skeletal muscle basement membrane fulfils several crucial functions during development and in the mature myotome and defects in its composition underlie certain forms of muscular dystrophy. (biologists.org)
- The cell membrane of a muscle cell is called the sarcolemma, and this membrane, like that of neurons, maintains a membrane potential. (eku.edu)
- DOMS is caused by localized damage to muscle tissue in the form of microtears in both the contractile proteins and surface membrane (i.e., sarcolemma) of the working muscles. (humankinetics.com)
- The cellular processes that transport channel proteins from the endoplasmic reticulum toward specified regions on the sarcolemma and, subsequently, remove them from the plasma membrane and direct them to the protein degradation machinery are commonly known as trafficking. (frontiersin.org)
- DMD, and BMD, which is the less severe form, are neuromuscular diseases caused by heritable mutations in the single dystrophin gene, which ultimately lead to progressive muscle weakness and degeneration due to destabilization of the sarcolemma (muscle cell membrane) and the resultant loss of muscle integrity. (prnewswire.co.uk)
- Understanding how ions and proteins interact with the surface of a cell membrane will enable us to determine how large macromolecular structures can be formed through protein association and ion-induced phase separation. (edu.au)
- A skeletal muscle cell is surrounded by a plasma membrane called the sarcolemma with a cytoplasm called the sarcoplasm. (lumenlearning.com)
- Satellite cells are located between the basement membrane and sarcolemma (cell membrane) of individual muscle fibers. (lumenlearning.com)
- A sarcolemma is the cell membrane, a sarcosome is a mitochondria, sarcoplasm is cytoplasm etc. (mindandmuscle.net)
- Each myofiber is an elongated syncytium, surrounded by the sarcolemma (plasmatic membrane) and containing myofibrils, which are made up of regularly and repeatedly organized contractile proteins (actin and myosin filaments). (jove.com)
- This gives it enough 'voltage' to reach its threshold which initiates an action potential on the muscle cell membrane (sarcolemma) which stimulates Ca2+ release from the sarcoplasmic reticulum to stimulate contraction (second messenger). (biology-online.org)
- Cellular Localization: Cell membrane, sarcolemma. (lsbio.com)
Fibers20
- We performed confocal immunofluorescence analysis of isolated sarcolemma that were mechanically peeled from skeletal fibers of mouse hindlimb muscle. (rupress.org)
- On the other hand, skeletal muscles are formed by morphological units referred to as muscle fibers . (wikipedia.org)
- Skeletal muscle fibers help support and move the body and are called syncytia - multinucleated structures formed by fusion of individual myoblasts during embryonic development. (wikipedia.org)
- In skeletal muscle, at the end of each muscle fiber, the outer layer of the sarcolemma combines with tendon fibers. (wikipedia.org)
- Individual muscle fibers (including the sarcolemma) are then surrounded by endomysium, a connective tissue . (newworldencyclopedia.org)
- The ability to form new fibers was verified by the presence of dMyHC positive regenerating fibers. (nature.com)
- Using these and other analyses, we present a model of lama2 function where fiber detachment external to the sarcolemma is mechanically induced, and retracted fibers with uncompromised membranes undergo subsequent apoptosis. (pnas.org)
- Individual muscle fibers are formed during development from the fusion of several undifferentiated immature cells known as myoblasts into long, cylindrical, multi-nucleated cells. (wikipedia.org)
- Adhesion of muscle fibers to the extracellular matrix (ECM) is essential for skeletal muscle development and integrity and is mediated by two protein complexes: the dystrophin glycoprotein complex (DGC) and complexes formed by members of the integrin superfamily. (biologists.org)
- Skeletal muscle cells form when many smaller progenitor cells lump themselves together to form long, straight, multinucleated fibers. (innerbody.com)
- In the adult muscle, SCs-quiescent under normal physiological conditions-are activated in response to trauma and are able to self-renew, proliferate, and differentiate to fuse to damaged fibers or form new myofibers ( 8 ). (asm.org)
- Expressed in muscle fibers accumulating in the costameres of myoplasm at the sarcolemma. (abcam.com)
- The chronic form of alcoholic skeletal myopathy is characterized by selective atrophy of Type II fibers and affects up to two thirds of all alcohol misusers. (bioscience.org)
- Myocytes, sometimes called muscle fibers, form the bulk of muscle tissue. (lumenlearning.com)
- Muscle fibers form from the fusion of myoblasts into multi-nucleated fibers called myotubes. (lumenlearning.com)
- Myoblasts that do not form muscle fibers dedifferentiate back into satellite (myosatellite) cells. (lumenlearning.com)
- These cells remain adjacent to a muscle fiber, situated between the sarcolemma and the endomysium (the connective tissue that divides the muscle fascicles into individual fibers). (lumenlearning.com)
- Muscle fibers are the bundles of cells that form the muscle. (mindandmuscle.net)
- Muscle biopsies in all four patients demonstrated 4-20% of fibers with internalized nuclei aligned in a basophilic ring (necklace) at 3 microm beneath the sarcolemma. (gu.se)
- Ultrastructural analysis revealed that palisade endings form IMCs, which were associated exclusively with multiply innervated global layer muscle fibers. (arvojournals.org)
Sarcoplasmic2
- The sarcoplasmic reticulum , a specialized type of smooth endoplasmic reticulum , forms a network around each myofibril of the muscle fiber. (wikipedia.org)
- together these three components form the triads that exist within the network of the sarcoplasmic reticulum, in which each T tubule has two terminal cisternae on each side of it. (wikipedia.org)
Extracellular matrix3
- Dystrophin localizes to the sarcolemma and stabilizes a complex of proteins (dystrophin-associated proteins, DAPs) that link the extracellular matrix to the actin cytoskeleton ( Ohlendieck, 1996 ). (biologists.org)
- Dystroglycan forms a transmembrane link between the extracellular matrix and the intracellular actin cytoskeleton through its interactions with laminin and dystrophin. (jneurosci.org)
- These two linkages represent the major connections between the actin cytoskeleton of the myofibre and the extracellular matrix, transmitting much of the force generated by muscle contraction across the sarcolemma (reviewed by Miner, 2008 ). (biologists.org)
Contraction12
- Triggers change in sarcolemma which triggers contraction Neuromuscular Junction Under control of the nervous system Control of Muscle Fiber Contraction How would severing the tendon attached to a muscle affect the ability of the muscle to move a body part? (prezi.com)
- One hypothesis is that the DGC protects the sarcolemma from contraction-induced damage. (rupress.org)
- Actin and myosin interact before muscle contraction, forming the contractile material actomyosin. (thefreedictionary.com)
- Muscle contraction occurs when there is stimulation in the form of an impulse or action potential from a motor nerve to muscle cell. (chegg.com)
- In the absence of dystrophin, almost all components of DGC is either lost or mislocalized, the DGC is rendered dysfunctional and, the sarcolemma is highly susceptible to damage during muscle contraction [ 8 ]. (iospress.com)
- The sarcomere is responsible for the striated appearance of skeletal muscle, and forms the basic machinery necessary for muscle contraction . (wikipedia.org)
- Because the SG complex plays a key role in the maintenance of sarcolemma integrity during muscle contraction, mutations in these genes lead to muscular dysfunction 3 . (nature.com)
- A major component of this extracellular structure is the laminin polymer, which assembles into a resilient meshwork that protects the sarcolemma during contraction. (biologists.org)
- In skeletal muscle, the BM surrounds each myofibre and provides the elasticity that enables the sarcolemma to withstand the mechanical stress of repeated contraction ( Sanes, 2003 ). (biologists.org)
- The balanced expression of many underlying channel proteins that transport ions with great selectivity at the sarcolemma of cardiomyocytes is crucial to maintain regular cardiac contraction throughout one's lifetime. (frontiersin.org)
- Mammalian adult skeletal muscles are formed by groups of fascicules of multinucleated muscle cells (myofibers) that are specialized for contraction. (jove.com)
- Sarcolemma contraction function? (studyblue.com)
Localizes to the sarcolemma1
- The DPC localizes to the sarcolemma and its disruption is associated with various forms of muscular dystrophy. (nih.gov)
Proteins8
- Protein Metabolism o Proteolysis- process of breaking down proteins o Protease- enzyme that breaks down proteins o Keto acids- class of breakdown products formed from the deamination oxidative acids. (majortests.com)
- Does not form homofilaments nor heterofilaments with other IF proteins. (genecards.org)
- Several of the muscular dystrophies, including DMD, BMD, CMD, and most forms of LGMD, are due to defects in the genes for a complex of muscle proteins. (encyclopedia.com)
- Two classes of proteins help form caveolae: Caveolins and Cavins. (pubmedcentralcanada.ca)
- The induction of MyoD on day 1 denoted the beginning of muscle regeneration and was followed by the reemergence of the embryonic forms of muscle contractile proteins, which peaked at day 7 . (physiology.org)
- While the idea of ion channels consisting of only pore-forming subunits persisted in the early days of ion channel research, it is now recognized that most ion channels function within macromolecular complexes, comprised not only of the pore-forming subunits, but also of associated auxiliary subunits, regulatory enzymes, and targeting proteins. (pubmedcentralcanada.ca)
- Thereafter, the role of non-pore-forming Nav-β-subunits channel proteins in trafficking of sodium and also the potassium channels and their importance in normal channel distribution within the cardiomyocyte are discussed by Edokobi and Isom . (frontiersin.org)
- The insulin and exercise signaling pathways are thought to converge at the level of the Rab GAP proteins TBC1D1 and AS160, which allow for the release of the GLUT4-containing vesicles from intracellular stores, translocation to the transverse tubules and sarcolemma, and an increase in glucose uptake. (nih.gov)
Cytoplasm1
- However, the release of Ca 2+ does not occur uniformly throughout the cytoplasm but often into a localized area before being transmitted to other regions of the cell in the form of Ca 2+ waves and oscillations to actively spread information within and between cells. (portlandpress.com)
Receptors2
- These postjunctional folds form what is referred to as the motor end-plate, which possesses acetylcholine receptors (AChRs) at a density of 10,000 receptors/micrometer2 in skeletal muscle. (primidi.com)
- What part of the sarcolemma contains acetylcholine receptors? (reference.com)
Actin8
- A population of γ-actin filaments was stably associated with sarcolemma isolated from normal muscle and displayed a costameric pattern that precisely overlapped with dystrophin. (rupress.org)
- However, costameric actin was absent from all sarcolemma isolated from dystrophin-deficient mdx mouse muscle even though it was localized to costameres in situ. (rupress.org)
- Our data demonstrate that the dystrophin complex forms a mechanically strong link between the sarcolemma and the costameric cytoskeleton through interaction with γ-actin filaments. (rupress.org)
- troponin, this triggers a shift in the position of tropomyosin, allowing the myosin heads to bind with actin forming cross-bridges. (scribd.com)
- The actin and myosin filaments in cardiac muscle exist as intertwined branches that form a conducting network for nerve impulses. (cliffsnotes.com)
- As non-striated muscle, the actin and myosin are not arranged into distinct sarcomeres that form orderly bands throughout the muscle cell. (wikidoc.org)
- Actin filaments attach to the sarcolemma by focal adhesions or attachment plaques and attach to other actin filaments via dense bodies (acting much like Z-lines in striated muscle). (wikidoc.org)
- These binding sites form a cross bridge with actin with the help of ADP/Pi. (biology-online.org)
Protein13
- However, it has not been determined whether the dystrophin complex can form a mechanically strong bond with any costameric protein. (rupress.org)
- Irregular shaped area inside a prokaryotic cell formed from the genetic material (DNA, RNA and protein complex) and serves as a nucleus. (brainscape.com)
- The accepted role of the protein Kv2.1 in arterial smooth muscle cells is to form K + channels in the sarcolemma. (pnas.org)
- Mutations in fukutin-related protein (FKRP) give rise to mild and more severe forms of muscular dystrophy. (biomedsearch.com)
- AChRs are localized by protein scaffolds at the postjunctional folds of the sarcolemma opposite the presynaptic terminals. (primidi.com)
- Mutations in the DMD gene result in the absence of a protein, dystrophin in the sarcolemma [ 3 ]. (iospress.com)
- Mutations in many protein components of DGC (such as dystrophin, sarcoglycans or dystroglycans) lead to various forms of muscular dystrophy and murine models with various dystrophic phenotypes, partly because certain components of DGC are more crucial in function than others [ 7 ]. (iospress.com)
- Within this group, sarcoglycanopathies are a subgroup caused by mutations in genes encoding the transmembrane protein sarcoglycan (SG) complex, located in the sarcolemma of striated muscles 2 . (nature.com)
- Dystrobrevin beta is thought to interact with syntrophin and the DP71 short form of dystrophin.This gene encodes dystrobrevin beta, a component of the dystrophin-associated protein complex (DPC). (acris-antibodies.com)
- Vid hjärtsvikt är ofta mängden desmin ökad, vilket i en del fall beror på mutationer i desmingenen eller mutationer i protein som hjälper till att bilda desminfilamenten. (lu.se)
- Becker muscular dystrophy (BMD), which is caused by mutations in the same gene, represents a milder form of dystrophinopathy due to higher degrees of functional dystrophin protein expression (5%-50% of normal levels). (jaoa.org)
- The autosomal recessive form (ARCNM) is caused by mutations in BIN1 , probably involving a partial loss-of-function as the protein level was found to be normal in previously described patients . (prolekare.cz)
- During the process of massaging brine in the outer layer of muscles contains increasing amounts of protein, becoming more and more viscous, forming the so-called adhesive capable of binding muscles during thermal processing. (media.pl)
Transverse tubules2
- These are generally located at the Z lines so that they form grooves and transverse tubules emanate. (wikipedia.org)
- The sarcolemma of myocytes contains numerous invaginations (pits) called transverse tubules which are usually perpendicular to the length of the myocyte. (lumenlearning.com)
Dystrophin-glycoprotein complex3
- Defects in components of the dystrophin-glycoprotein complex (DGC) are responsible for different forms of muscular dystrophies. (nature.com)
- Genetic defects in a number of components of the dystrophin-glycoprotein complex (DGC) lead to distinct forms of muscular dystrophy. (rupress.org)
- Major forms of muscular dystrophies are caused by the abnormalities of the dystrophin glycoprotein complex (DGC) that plays crucial roles as a structural unit and scaffolds for signaling molecules at the sarcolemma. (mdpi.com)
Penetrate deep1
- Certain regions of the sarcolemma penetrate deep into the cell. (wikipedia.org)
Targeting of nNOS1
- This targeting of nNOS to the sarcolemma enabled nNOS to improve blood flow through the muscles during exercise. (medicalnewstoday.com)
Myofibrils1
- Myofibrils run parallel to the myocyte and typically run for its entire length, attaching to the sarcolemma at either end. (lumenlearning.com)
Enclosed in a sheath2
- The bundles are then grouped together to form muscle, which is enclosed in a sheath of epimysium. (newworldencyclopedia.org)
- This type of muscle is composed of numerous cylindrically shaped bundles of cells, each enclosed in a sheath called the sarcolemma. (thefreedictionary.com)
Neuromuscular1
- Component of the dystrophin-associated glycoprotein complex which accumulates at the neuromuscular junction (NMJ) and at a variety of synapses in the peripheral and central nervous systems and has a structural function in stabilizing the sarcolemma. (abcam.com)
Myocytes4
- In myocytes, sarcomeres adhere to the sarcolemma via costameres, which align at Z-discs and M-lines. (wikipedia.org)
- Myocytes develop from myoblasts to form muscles in a process known as myogenesis . (wikipedia.org)
- In cardiac myocytes this forms a scalloped surface. (wikipedia.org)
- We found that amylin deposition negatively affects cardiac myocytes by inducing sarcolemmal injury, generating reactive aldehydes, forming amylin-based adducts with reactive aldehydes, and increasing synthesis of the proinflammatory cytokine interleukin-1β (IL-1β) independently of hyperglycemia. (diabetesjournals.org)
Cell7
- Activated DHPR's open, forming a channel, that allows Ca2+ to pass into the cell. (wikipedia.org)
- The cells of the inner cell mass (embryoblast), known as human embryonic stem cells (hESCs), differentiate to form four structures: the amnion, the yolk sac, the allantois, and the embryo itself. (lumenlearning.com)
- The first differentiation of the hESCs that form the embryo proper, is in three cell types known as the germ layers: the ectoderm , the mesoderm , and the endoderm . (lumenlearning.com)
- A myoblast is a type of embryonic progenitor cell that differentiates to form muscle cells. (lumenlearning.com)
- indeed, the myonuclei, which are located at the periphery of the myofiber, under the sarcolemma, are arrested in the G0 phase of the cell cycle and are unable to proliferate 1,2 . (jove.com)
- The sarcolemma possess microdomains specialized to cell-signaling events and ion channels called caveolae. (wikidoc.org)
- Moreover, they absorb water more effectively and faster diffusion is facilitated through the increased migration of curing brine components as a result of the disrupted cell structure and the ruptured muscle fiber sarcolemma . (media.pl)
Connective tissue3
- Over the layer of epimysium is fascia, a sheet of connective tissue that helps maintain form. (newworldencyclopedia.org)
- As collateral circulation enlarges, it brings fibroblasts, which form a connective tissue scar within the area of infarction. (thefreedictionary.com)
- They are bound together by perimysium, a sheath of connective tissue, into bundles called fascicles, which are in turn bundled together to form muscle tissue. (lumenlearning.com)
Molecules1
- This 30-nanometer space forms the synaptic cleft through which signaling molecules are released. (primidi.com)
Cardiac muscle1
- Recent evidence suggests the involvement of vascular smooth muscle DGC in skeletal and cardiac muscle pathology in some forms of sarcoglycan-deficient limb-girdle muscular dystrophy. (nih.gov)
Muscles2
- LGMD2D is caused by mutations in the gene encoding α-sarcoglycan (α-SG), a dystrophin-associated glycoprotein that plays a key role in the maintenance of sarcolemma integrity in striated muscles. (nature.com)
- The mesoderm forms the skeleton and muscles, heart and circulatory system, urinary and reproductive systems, and connective tissues inside the body. (lumenlearning.com)
Integrity1
- In skeletal muscle, this link provides structural integrity to the sarcolemma ( Ervasti and Campbell, 1993 ). (jneurosci.org)
Smooth2
- Smooth muscle cells respond to Ins P 3 -generating (sarcolemma-acting) neurotransmitters and hormones by releasing Ca 2+ from the internal store. (portlandpress.com)
- Here, we report that a novel form of a local Ca 2+ signaling in arterial smooth muscle is linked to the development of angiotensin II-induced hypertension. (ahajournals.org)
Myofibers2
- In response to muscle damage or under increased workload these cells activate, migrate into damaged sites, proliferate, and fuse with existing myofibers or with each other to form new myofibers, whereas a small portion of these cells returns into quiescence [11, (thefreelibrary.com)
- Following an injury or disease, SCs become activated, proliferate, migrate to the damaged areas, and eventually differentiate, giving rise to newly forming myofibers 5 . (jove.com)
Structural1
- There is an extensive literature addressing the subcellular structural alterations in HF, 4 - 8 with some variability in structural and functional remodeling in differing forms of experimental and human HF. (ahajournals.org)
Synaptic1
- Maturation and stabilization of AChR clusters ensure proper synaptic development, which forms the basis for nerve-evoked muscle contractibility. (jneurosci.org)
Glycoprotein complex1
- Stabilization of the soluble, cleaved, trimeric form of the envelope glycoprotein complex of human immunodeficiency virus type 1. (semanticscholar.org)
Muscular dystrophies2
- It is the most variable of the muscular dystrophies, and there are as of 2004 several different forms of the disease recognized. (encyclopedia.com)
- Duchenne muscular dystrophy (DMD) is the most common and fatal form of muscular dystrophies with an incidence of 1 in 5,000 boys [ 1, 2 ]. (iospress.com)
Mutations4
- Mutations in the human laminin α 2 ( LAMA2 ) gene result in the most common form of congenital muscular dystrophy (MDC1A). (pnas.org)
- While it is widely-accepted that mutations in ion channel pore-forming genes underlie a number of cardiac arrhythmias, current research is now focusing on the roles of auxiliary subunits in the development of arrhythmia syndromes. (pubmedcentralcanada.ca)
- The X-linked and dominant CNM forms result from mutations in the phosphoinositide phosphatase myotubularin ( MTM1 ) and the large GTPase dynamin 2 ( DNM2 ), respectively , . (prolekare.cz)
- However, mutations in MTM1 have also been recognized as the underlying cause of "atypical" forms of XLMTM in newborn boys, female infants, female manifesting carriers and adult men. (gu.se)
Limb-Girdle Mu1
- Intriguingly, two other forms of limb-girdle muscular dystrophy are possibly caused by perturbation of sarcolemma repair mechanisms. (nih.gov)
Calcium3
- What would you expect to happen to a resting skeletal muscle if the sarcolemma suddenly became very permeable to calcium ions? (prezi.com)
- Muscle tissue is formed in the mesoderm layer of the embryo in response to signals from fibroblast growth factor, serum response factor, and calcium. (lumenlearning.com)
- The sliding filaments require that calcium ions and energy in the form of ATP be available. (cliffsnotes.com)
Amino Acid1
- o Oxidative deamination- taking an amino group off of an amino acid and form ammonia and a keto acid Oxidative deamination: take one amino acid and make it into another one and can basically break down amino acids that ultimately wind up being how to get rid of excess amino acids in the system. (majortests.com)
Syntrophin3
- Palmitoylated dystrobrevin restored α-syntrophin and aquaporin-4 (AQP4) to the mdx sarcolemma but was unable to recruit β-dystroglycan or the sarcoglycans. (biologists.org)
- Despite restoration of sarcolemmal α-syntrophin, neuronal nitric oxide synthase (nNOS) was not localized to the sarcolemma, suggesting that nNOS requires both dystrophin and α-syntrophin for correct localization. (biologists.org)
- Dystrobrevin beta is thought to interact with syntrophin and the DP71 short form of dystrophin. (novusbio.com)
Cardiomyocytes1
- Cardiomyocytes are the cells that form the chambers of the heart, and have a single central nucleus . (wikipedia.org)
Localization1
- We report here on the development of a new in vitro high-throughput screening assay that allows the monitoring of the proper localization of the most prevalent mutant form of α-SG (R77C substitution). (nature.com)
Functional2
- Expression of palmitoylated α-dystrobrevin prevented the muscular dystrophy observed in the α-dystrobrevin-null mice, demonstrating that the altered form of α-dystrobrevin was functional. (biologists.org)
- Moreover, regional differences in action potential shape within the heart allude to the tuning and fine tuning of functional ion channel expression at the sarcolemma. (frontiersin.org)
Muscle tissue4
- Striated, or skeletal, muscle forms the bulk of the body's muscle tissue and gives the body its general shape. (thefreedictionary.com)
- It is a form of striated muscle tissue , which is under the voluntary control of the somatic nervous system . (wikipedia.org)
- In the presence of fibroblast growth factor, myoblasts fuse into multi-nucleated mytotubes, which form the basis of muscle tissue. (lumenlearning.com)
- Collections of cells form muscle tissue. (mindandmuscle.net)
Differentiate1
- Satellite cells are able to differentiate and fuse to augment existing muscle fibres and to form new fibres. (bionity.com)
Attachment1
- Secondly, it forms an attachment via integrins to subsarcolemmal focal adhesion complexes. (biologists.org)
Tissue1
- Specifically, the divergence of single-celled life forms into organisms composed of multiple cells types with specific functions required intercellular/intracellular communication, tissue synchronicity, homeostatic measures, and movement. (pubmedcentralcanada.ca)