Results 281 to 290 of about 72,501 (324)
The toxic effect of R350P mutant desmin in striated muscle of man and mouse
Acta Neuropathologica, 2014 Mutations of the human desmin gene on chromosome 2q35 cause autosomal dominant, autosomal recessive and sporadic forms of protein aggregation myopathies and cardiomyopathies.
Christoph S Clemen +2 more
exaly +2 more sources
Some of the next articles are maybe not open access.
Related searches:
Related searches:
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.
openaire +2 more sources
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.
openaire +2 more sources
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.
openaire +1 more source
Imbalances in protein homeostasis caused by mutant desmin
Neuropathology and Applied Neurobiology, 2018We investigated newly generated immortalized heterozygous and homozygous R349P desmin knock‐in myoblasts in conjunction with the corresponding desminopathy mice as models for desminopathies to analyse major protein quality control processes in response ...
L. Winter +16 more
semanticscholar +1 more source
Bulletin of the Academy of Sciences of the USSR Division of Chemical Science, 1987
1. A new mechanism is proposed for the dehydration of desmine, according to which dissociation of some of the water molecules coordinated with the calcium cations occurs to form hydroxyls after a certain stage. 2. It has been established that the processes of hydroxyl formation and dehydroxylation are the reasons for the irreversible ...
J. I. Mirzai +4 more
openaire +1 more source
1. A new mechanism is proposed for the dehydration of desmine, according to which dissociation of some of the water molecules coordinated with the calcium cations occurs to form hydroxyls after a certain stage. 2. It has been established that the processes of hydroxyl formation and dehydroxylation are the reasons for the irreversible ...
J. I. Mirzai +4 more
openaire +1 more source
Desmin in muscle and associated diseases: beyond the structural function
Cell and Tissue Research, 2014Karim Hnia +2 more
exaly +2 more sources
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.
openaire +2 more sources
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
openaire +2 more sources
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.
openaire +1 more source
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.
openaire +1 more source
Desmin‐related neuromuscular disorders
Muscle & Nerve, 1995AbstractDesmin, the intermediate filament protein of skeletal muscle fibers, cardiac myocytes, and certain smooth muscle cells, is a member of the cytoskeleton linking Z‐bands with the plasmalemma and the nucleus. The pathology of desmin in human neuromuscular disorders is always marked by increased amounts, diffusely or focally.
openaire +2 more sources