The dysfunction of the proteins involved in costameres contributes to some muscular diseases, including muscular dystrophies and cardiomyopathies. (wikipedia.org)
Costameres are clearly important for normal muscle function because several constituent proteins are the primary sites of defect in human muscular dystrophies and dilated cardiomyopathies. (grantome.com)
Dystrophin3
Costameres are highly complex networks of proteins and glycoproteins, and can be considered as consisting of two major protein complexes: the dystrophin-glycoprotein complex (DGC) and the integrin-vinculin-talin complex. (wikipedia.org)
For instance, mechanical tension is critical in regulating costameric protein expression, stability, and organization, and dystrophin deficient costameres may sense increased mechanical stress and attempt to compensate with filament recruitment. (wikipedia.org)
KRT8 together with KRT19, help to link the contractile apparatus to dystrophin at the costameres of striated muscle. (avivasysbio.com)
Protein assemblies2
Costameres are sub-sarcolemmal protein assemblies circumferentially aligned in register with the Z-disk of peripheral myofibrils. (wikipedia.org)
Costameres are subsarcolemmal protein assemblies in striated muscle cells that circumferentially align in register with the Z disk of peripheral myofibrils and physically couple force-generating sarcomeres with the sarcolemma. (grantome.com)
Cytoskeletal2
The vinculin and talin components of the integrin-vinculin-talin complex are cytoskeletal proteins physically anchored to the costamere as a whole via the integrin components, which are transmembrane proteins that interact directly with filamin C of the Z disk. (wikipedia.org)
It has been suggested that synemin could function as a linker between different cytoskeletal components based on the fact that it interacts with several proteins involved in the organization of the costameres, neuromuscular and myotendinous junctions within striated muscle cells. (biomedcentral.com)
Sarcolemma5
The costamere is a structural-functional component of striated muscle cells which connects the sarcomere of the muscle to the cell membrane (i.e. the sarcolemma). (wikipedia.org)
Costameres also transmit forces in the opposite direction, transmitting the forces of external mechanical stress from the sarcolemma to the Z-disk. (wikipedia.org)
Costameres are also involved in protecting the relatively weak and labile sarcolemma from the mechanical stresses of contraction and stretching. (wikipedia.org)
We have learned that the organization of structural proteins at the sarcolemma, and especially at costameres , is affected in several forms of muscular dystrophy. (blochlab.com)
Cav3 appears in the cardiomyocyte sarcolemma, intercalated discs and T-tubules and was recognized in the costameres, rib-like perisarcolemmal multiprotein complexes that align with Z disks and T-tubules and function in cell adhesion, stretch-sensing and pressure transmission [28], [29]. (sdfca.org)
and cytoplasmic FRG-1, associated with the Z-disk and costamere-like structures known as dense bodies. (illinois.edu)
Desmin IFs are present throughout smooth, cardiac and skeletal muscle cells, but can be more concentrated in some particular structures, such as dense bodies, around the nuclei, around the Z-line or in costameres. (biologicalworld.com)
Mechanical2
Several studies have suggested that costameres are responsive to mechanical, electrical, and chemical stimuli. (wikipedia.org)
Finally, we will assess the role of y-actin in costamere assembly and mechanical function by characterizing new lines of mice where it is specifically ablated in striated muscle. (grantome.com)
Complex2
The sarcoglycans of the DGC and the integrins of the integrin-vinculin-talin complex attach directly to filamin C, a component of the Z-disk, linking these protein complexes of costameres to complexes of the Z-disk. (wikipedia.org)
Restated, filamin C physically links the two complexes that constitute the costamere to sarcomeres by interacting with the sarcoglycans in the DGC and the integrins of the integrin-vinculin-talin complex. (wikipedia.org)
Link3
Link to all annotated objects annotated to costamere. (cyverse.org)
Link to all direct and indirect annotations to costamere. (cyverse.org)
Link to all direct and indirect annotations download (limited to first 10,000) for costamere. (cyverse.org)
Myotendinous junctions1
The 71 integrin is hugely expressed in skeletal muscle fibers and particularly localizes at costameres and myotendinous junctions [142], exactly where it physically connects the ECM towards the sarcomeric contractile apparatus [143]. (squalene-epoxidase.com)
Sarcomere1
Moreover, the costameres at the cell surface interact with the repeating Z-disc component of the intracellular sarcomere, the contractile unit in muscle, through the actin cytoskeleton. (nature.com)
Cell membrane1
As Costamere are responsible for linking of internal cytoskeletal system of each muscle fibers to extracellular matrix of collagen and laminin through cell membrane. (atomictherapy.org)
Protein4
Costameres are sub-sarcolemmal protein assemblies circumferentially aligned in register with the Z-disk of peripheral myofibrils. (wikipedia.org)
The sarcoglycans of the DGC and the integrins of the integrin-vinculin-talin complex attach directly to filamin C, a component of the Z-disk, linking these protein complexes of costameres to complexes of the Z-disk. (wikipedia.org)
We identified an interaction of paxillin with the vinexin adaptor protein family member ponsin in nascent costameres during muscle differentiation, which is mediated by an interaction of the second src homology domain 3 (SH3) domain of ponsin with the proline-rich region of paxillin. (ox.ac.uk)
Here, we describe the characterization of a direct, downstream target gene for the MEF2A transcription factor encoding a large, muscle-specific protein that localizes to the costamere in striated muscle. (elsevierpure.com)
Interact2
The vinculin and talin components of the integrin-vinculin-talin complex are cytoskeletal proteins physically anchored to the costamere as a whole via the integrin components, which are transmembrane proteins that interact directly with filamin C of the Z disk. (wikipedia.org)
Paxillin and ponsin interact in nascent costameres of muscle cells. (ox.ac.uk)
Proteins1
The dysfunction of the proteins involved in costameres contributes to some muscular diseases, including muscular dystrophies and cardiomyopathies. (wikipedia.org)
Structural-functional1
Costamere is an integral component in maintaining structural-functional integrity of striated muscle cells. (atomictherapy.org)
Physically1
Restated, filamin C physically links the two complexes that constitute the costamere to sarcomeres by interacting with the sarcoglycans in the DGC and the integrins of the integrin-vinculin-talin complex. (wikipedia.org)
Component1
15. Distribution of formins in cardiac muscle: FHOD1 is a component of intercalated discs and costameres. (nih.gov)
Our findings demonstrate that myospryn functions directly downstream of MEF2A at the costamere in striated muscle potentially playing a role in myofibrillogenesis. (elsevierpure.com)
Studies1
Several studies have suggested that costameres are responsive to mechanical, electrical, and chemical stimuli. (wikipedia.org)
Functions1
Costameres have several primary functions. (wikipedia.org)