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Desmin at myotendinous junctions
Experimental Cell Research, 1992Myofibrils are linked to the cell membrane at myotendinous junctions located at the ends of muscle fibers, and at costameres, sites positioned periodically along lateral surfaces of muscle cells. Both of these sites are enriched in proteins that link active components of myofibrils to the cell membrane.
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Abnormal desmin protein in myofibrillar myopathies caused by desmin gene mutations
Annals of Neurology, 2001AbstractMuscle proteins were extracted in various sodium dodecyl sulfate buffers from 6 patients with myofibrillar myopathy (MFM) and previously identified with mutations in the desmin gene (desmin myopathy; DesM), 6 with MFM without mutations, and 14 disease controls to search for alterations in biochemistry and solubility of mutated desmin filaments.
M, Li, M C, Dalakas
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Desmin Filaments and Desmin-Related Myopathy
2015Desmin is the major intermediate filament protein in muscle tissue. In skeletal and cardiac muscle, desmin filaments distribute in the intermyofibrillar space to link adjacent myofibrils to each other at the z disc level, to the sarcolemma via costameres and sarcoglycan-dystroglycan complexes, and insert into to the nuclear envelope.
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Desmin Myopathy, a Skeletal Myopathy with Cardiomyopathy Caused by Mutations in the Desmin Gene
New England Journal of Medicine, 2000Myofibrillar myopathies, often referred to as desmin-related myopathies, are a heterogeneous group of inherited or sporadic distal-onset skeletal myopathies associated with cardiomyopathy. Among the myofibrillar proteins that characteristically accumulate within the muscle fibers of affected patients, the one found most consistently is desmin, a muscle-
M C, Dalakas +5 more
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Current Opinion in Neurology, 1997
Desmin-related myopathies are marked by accumulation of desmin, which is often familial and associated with cardiomyopathy. When multifocal this excess is characterized by inclusions such as cytoplasmic or spheroid bodies, when disseminated the excess is called granulofilamentous material.
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Desmin-related myopathies are marked by accumulation of desmin, which is often familial and associated with cardiomyopathy. When multifocal this excess is characterized by inclusions such as cytoplasmic or spheroid bodies, when disseminated the excess is called granulofilamentous material.
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Structural characteristics of the desmin protofilament
Journal of Ultrastructure Research, 1984Biochemical investigations of intermediate filaments in soluble or partially assembled forms are often difficult to perform due to the unusual insolubility of most types of intermediate filaments. However, desmin is soluble in 10 mM Tris. The structure of partially soluble native desmin was studied by gel-filtration chromatography and electron ...
L, Milam, H P, Erickson
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Desmin and vimentin in regenerating muscles
Muscle & Nerve, 1992AbstractDesmin is a normal constituent of skeletal muscle fibers; vimentin is contained in myoblasts and connective tissue cells. The intracellular localization of both intermediate filament proteins in regenerating rat muscles was investigated by immunohisto‐ and immunocytochemistry. Necrosis was induced by hot Ringer solution.
Bornemann, A., Schmalbruch, Henning
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1999
Abstract Desmin belongs to the intermediate filament family of proteins. Protein sequence homology and intron placement of the desmin gene classify desmin as a type Ill IF protein, together with vimentin, peripherin, and giial fibrillary acidic protein.
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Abstract Desmin belongs to the intermediate filament family of proteins. Protein sequence homology and intron placement of the desmin gene classify desmin as a type Ill IF protein, together with vimentin, peripherin, and giial fibrillary acidic protein.
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Desmin pathology in neuromuscular diseases
Virchows Archiv B Cell Pathology Including Molecular Pathology, 1993Desmin is an intermediate filament protein that in striated muscle is normally located at Z-bands, beneath the sarcolemma, and prominently at neuromuscular junctions. It is abundant during myogenesis and in regenerating fibers, but decreases in amount with maturation; in regenerating and denervated muscle fibers it is co-expressed with vimentin ...
H H, Goebel, A, Bornemann
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Osteogenic Melanoma With Desmin Expression
The American Journal of Dermatopathology, 2017Background: Osteogenic differentiation is rarely seen in melanomas, when it occurs it is mainly in acral lesions. Methods: We report a case of an osteogenic melanoma in a 49-year-old woman who presented with a pigmented lesion in the subungueal region of her left hallux.
Flávia, Trevisan +8 more
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