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[The localization of the components of the extracellular matrix and cytoskeleton in myocardial biopsies in alcoholic cardiomyopathy].

Distribution of I, III, IV and V-type collagen, fibronectin, vimentin, filamin, and desmin in myocardial biopsy specimens of 8 patients with alcoholic cardiomyopathy was studied by indirect immunofluorescence. It is found that all types of collagen and fibronectin participate in formation of diffuse cardiosclerosis while small cardiosclerosis is formed by interstitial types of collagen and to a lower extent of fibronectin. Filamin and desmin distribution in cardiomyocytes proves deformation and destruction of Z-lines and intercalated discs.

Cell cultures of human ciliary muscle: growth, ultrastructural and immunocytochemical characteristics.

Primary ciliary muscle cell cultures derived from human donors (16-91 years) were established and characterized by comparing them with ciliary muscle in tissue sections using immunocytochemical and ultrastructural methods. Monoclonal antibodies against desmin, vimentin, alpha-actinin, smooth muscle (sm) specific alpha-actin and von Willebrand factor were used. In tissue sections of the ciliary body, ciliary muscle cells, vascular muscle cells, pericytes, endothelial cells and fibroblasts stain for vimentin. Both types of muscle cells and the pericytes stain for alpha-sm-actin, but only ciliary…

Cytoskeletal and contractile proteins in detrusor smooth muscle from bladders with outlet obstruction--a comparative study in rat and man.

Detrusor biopsies were obtained from patients with urinary outlet obstruction due to prostatic enlargement and from age-matched control patients. The relative amounts of actin and myosin and their isoforms, as well as desmin and filamin were determined and compared with corresponding results from bladders from control rats and rats with 10 days of experimental outlet obstruction of the urinary bladder. In the human control detrusor the actin/myosin ratio was similar to that in the control rat. The isoform distribution of the myosin heavy chains differed between man and rat. In the biopsies fro…

Clathrin plaques and associated actin anchor intermediate filaments in skeletal muscle.

Clathrin plaques are stable features of the plasma membrane observed in several cell types. They are abundant in muscle, where they localize at costameres that link the contractile apparatus to the sarcolemma and connect the sarcolemma to the basal lamina. Here, we show that clathrin plaques and surrounding branched actin filaments form microdomains that anchor a three-dimensional desmin intermediate filament (IF) web. Depletion of clathrin plaque and branched actin components causes accumulation of desmin tangles in the cytoplasm. We show that dynamin 2, whose mutations cause centronuclear my…

Stromal-Derived IL-8 Promotes M2 Macrophage Polarization via the RhoA/MRTF-A/SRF Transcriptional Axis to Impair CD8+ T Cell Cytotoxicity in Lung Cancer.

BACKGROUND: Building upon our previous finding that tumor-derived lactate activates DESMIN+ cancer-associated fibroblasts (CAFs) to secrete interleukin-8 (IL-8), this study investigates the downstream IL-8/C-X-C motif chemokine receptor 2 (CXCR2) axis in CAF-mediated macrophage polarization and lung cancer (LC) progression. METHODS: Orthotopic and subcutaneous tumor models were established in immunocompetent and immunodeficient mice using LC, CAFs, or murine tumor organoids (MTO). Global and myeloid-specific Cxcr2 knockout models, in vitro co-cultures (macrophages, CD8+ T cells, and tumor cel…

Nodular fasciitis of the vulva: a challenging histopathologic diagnosis supported by the detection of USP6 gene rearrangement.

Nodular fasciitis involving the vulva on physical examination can mimic a Bartholin gland lesion, and histologically can have overlapping features with more ominous mesenchymal pathologies. We describe a case in which a 52-year-old perimenopausal woman presents with a vulvar mass. After an initial biopsy and later excision, the myofibroblastic lesion was noted to have areas of differing cellularity, with compact nodule formations at the periphery. Immunohistochemical staining showed lesional cells to be positive for desmin, estrogen receptor, progesterone receptor, and smooth muscle actin, and…

Характеристика неимперативных Myofibroblasts Stellate Celle или Mesenchymal Cell Production в D-Galactosamin-приводимой печени в крысах.

Гепатотоксичность, вызванная D-галактозамином (D-GalN), похожа на гепатотоксичность острого вирусного гепатита. В ходе этого исследования были исследованы печенитические мезенхимальные клетки, включая печенитические клетки (HSC) и миофибробласты, вызванные гепатотоксичностью D-GalN. Крысы, вводимые D-GalN (800 мг/кг веса тела, один раз внутриперитонально), исследовались при однократном впрыске (PSI) в течение 8 часов и дней с 1 по 5. Лезионы, состоящие из гепатоцитового некроза и репаративного фиброза, присутствовали диффузно или в фокусе печени в дни 1 и 2 PSI, а затем травмы, полученные в дн

Совпадение в аннотации оригинала
Lesions of D-galactosamine (D-GalN)-induced hepatotoxicity resemble those of human acute viral hepatitis. This study investigated hepatic me … ays 1 and 2, and then the injury recovered on PSI days 3 and 5. Myofibroblasts expressing vimentin, desmin, and α-smooth muscle actin (α-SMA) were present in the lesions. Double immunofluorescence showed that myofibroblasts reacted simultaneously to vimentin/α-SMA, desmin/α-SMA, and desmin/vimentin; furthermore, myofibroblasts reacting to vimentin, desmi …

Histogenesis of alveolar soft part sarcoma. An immunohistochemical and biochemical study.

In order to clarify the histogenesis of alveolar soft part sarcoma (ASPS), an immunohistochemical and biochemical study was performed on three cases. The immunohistochemical study indicated the presence of actin, desmin, vimentin, and Z-protein in all cases. On the other hand, intermediate filaments other than desmin and vimentin were not detected immunohistochemically. The presence of desmin and Z-protein strongly suggests the myogenic character of this tumor. As to whether ASPS shows striated muscle differentiation or smooth muscle differentiation, the immunohistochemical absence of myoglobi…

Consequences of plectin ablation on the various intermediate filament systems in skeletal muscle.

Plectin, a highly versatile and multifunctional cytolinker, acts as a central connector of the intermediate filament (IF) and other cytoskeletal systems. In skeletal muscle, plectin orchestrates and anchors the extrasarcomeric desmin filament network to sites of strategic importance and thereby substantially contributes to its fundamental biomechanical properties. Lack of plectin in skeletal muscle leads to a faulty organization of the desmin IFs, thereby inflicting a reduced mechanical stress tolerance and a progressive myopathic process. Accordingly, the morphological hallmark of the skeleta…

Immunoblotting of contractile and cytoskeletal proteins of canine basilar artery in vasospasm.

Vasospasm was produced in the canine basilar arteries by a two-hemorrhage method, and voltage- and receptor-dependent contractions of the normal canine basilar arteries were induced by local applications of potassium chloride (KCI) and serotonin, respectively, after transclival exposure. Actin, myosin, desmin, filamin, talin, vinculin, and alpha-actinin in the basilar artery were studied by immunoblotting. The immunoblots showed a decrease or loss in immunoreactivity of some native proteins and generation of protein fragments, smaller in size than native proteins, in spastic, KCI, and serotoni…

CD10 imunostaining does not distinguish endometrial carcinoma invading myometrium from carcinoma involving adenomyosis.

The distinction of involvement of adenomyosis by endometrial carcinoma from endometrial carcinoma invading the myometrium can at times be di … Hopkins Hospital and consultation files of one of the authors (R.J.K.) and immunostained for CD10, desmin, and caldesmon. The pattern of staining was compared with five cases in which carcinoma was confined to adenomyosis. As a control, 14 cases of adenomyosis unassociated with carcinoma were included in the analysis. All 22 endometrial carcinomas that …

Glial fibrillary acidic protein--a cell type specific marker for Ito cells in vivo and in vitro.

BACKGROUND/AIMS: Glial fibrillary acidic protein is an intermediate filament first identified in the brain in astrocytes. This study examines glial fibrillary acidic protein immunoreactivity in normal and damaged rat livers. Glial fibrillary acidic protein-gene-expression in Ito cells, endothelial cells, Kupffer cells, and hepatocytes is also analyzed. METHODS: Sequential cryostat sections from normal, as well as acutely or chronically CC14 damaged rat livers were analyzed by immunostaining for the presence of glial fibrillary acidic protein and desmin. Glial fibrillary acidic protein-express…

The effect of vitamin A on CCl4-induced hepatic injuries in rats: a histochemical, immunohistochemical and ultrastructural study.

In this study, we aimed to investigate the effect of vitamin A on the transformation of the Ito cells to fibrogenic form and suppression of the development of fibrosis. Carbon tetrachloride intoxication was performed on rats for 2, 8, 12 or 20 weeks and 5x10(4) IU vitamin A (as retinol palmitate) was injected subcutaneously once every 4 weeks. Ito cells were detected by gold chloride impregnation, as well as desmin and alpha-smooth muscle actin (alpha-SMA) immunohistochemistry. Additionally, all groups were examined ultrastructurally. The number of Ito cells that were labelled positively with …

Using of immunocytochemistry in differential diagnosis of neoplasms of serosal cavities in dogs.

The presence of tumor within the serosal cavities, often connected with accumulation of serosal effusion, is a quite common problem in the small animal veterinary medicine. The first step in diagnosis of such cases is cytopathological examination. The aim of the present study was to evaluate the usefulness of cytology and immunocytochemistry, using commercially available antibodies (anti-cytokeratin, anti-vimentin, and anti-desmin), in differential diagnosis of malignant tumors located within serosal cavities in dogs. The final cytological diagnosis of carcinoma/adenocarcinoma, sarcoma, and me…

A Novel L1 Linker Mutation in DES Resulted in Total Absence of Protein.

Desminopathies (MIM*601419) are clinically heterogeneous, manifesting with myopathy and/or cardiomyopathy and with intra-sarcoplasmic desmin-positive deposits. They have either an autosomal dominant (AD) or recessive (AR) pattern of inheritance. Desmin is a crucial intermediate filament protein regulating various cellular functions in muscle cells. Here, we report a 13-year-old girl, born of second-degree consanguineous parents, with normal developmental milestones, who presented with dilated cardiomyopathy, respiratory insufficiency and predominant distal upper limb weakness. A striking featu…

Severe cardiac phenotype with right ventricular predominance in a large cohort of patients with a single missense mutation in the DES gene.

BACKGROUND: Desmin-related myopathy is a clinically heterogenous group of disorders encompassing myopathies, cardiomyopathies, conduction disease, and combinations of these disorders. Mutations in the gene encoding desmin (DES), a major intermediate filament protein, can underlie this phenotype. OBJECTIVE: The purpose of this study was to investigate the clinical and pathologic characteristics of 27 patients from five families with an identical mutation in the head domain region (p.S13F) of desmin. METHODS/RESULTS: All 27 carriers or obligate carriers of a p.S13F DES founder mutation demonst…

Intermediate filament diseases: desminopathy.

Desminopathy is one of the most common intermediate filament human disorders associated with mutations in closely interacting proteins, desmin and alphaB-crystallin. The inheritance pattern in familial desminopathy is characterized as autosomal dominant or autosomal recessive, but many cases have no family history. At least some and likely most sporadic desminopathy cases are associated with de novo DES mutations. The age of disease onset and rate of progression may vary depending on the type of inheritance and location of the causative mutation. Typically, the illness presents with lower and …

Deficiency in nebulin repeats of sarcomeric nebulette is detrimental for cardiomyocyte tolerance to exercise and biomechanical stress.

The actin-binding sarcomeric nebulette (NEBL) protein provides efficient contractile flexibility via interaction with desmin intermediate filaments. NEBL gene mutations affecting the nebulin repeat (NR) domain are known to induce cardiomyopathy. The study aimed to explore the roles of NEBL in exercise and biomechanical stress response. We ablated exon3 encoding the first NR of Nebl and created global Neblex3-/ex3- knockout mice. Cardiac function, structure, and transcriptome were assessed before and after a 4-wk treadmill regimen. A Nebl-based exercise signaling network was constructed using s…

AAV-based TCAP delivery rescues mitochondria dislocation in limb-girdle muscular dystrophy R7.

Limb-girdle muscular dystrophy R7 is a rare genetic disease caused by homozygous or compound heterozygous variants in the titin-cap (TCAP) g … Through our in vitro experiments, we discovered that telethonin aids in preserving the integrity of desmin by preventing truncation at the N-terminus. Additionally, telethonin combined with desmin and co-localized at the Z-disc. Research has shown that the Tcap gene plays a crucial role in controlling the desmin cytoskeleton organization. The absence of …

Alterations in intermediate filaments expression in disc cells from the rat temporomandibular joint following exposure to continuous compressive force.

The articular disc in the temporomandibular joint (TMJ) that serves in load relief and stabilizing in jaw movements is a dense collagenous t … xpression of these intermediate filaments and an additional intermediate filament - muscle-specific desmin - in the disc cells of the TMJ disc using a rat experimental model. The rats wore an appliance that exerts a continuous compressive load on the TMJ. The experimental period with the appliance was 5 days as determined by previous studies, after which …