scholarly journals Ultrastructural analysis of small blood vessels in skin biopsies in CADASIL

2008 ◽  
Vol 60 (4) ◽  
pp. 573-580 ◽  
Author(s):  
Vesna Lackovic ◽  
M. Bajcetic ◽  
Nadezda Sternic ◽  
V. Kostic ◽  
Jasna Zidverc ◽  
...  
Keyword(s):  
Electron Microscopy ◽  
Smooth Muscle ◽  
Blood Vessels ◽  
Autosomal Dominant ◽  
Specific Marker ◽  
Dense Bodies ◽  
Skin Biopsies ◽  
Artery Disease ◽  
Skin Blood Vessels ◽  
The Brain

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited small- and medium-artery disease of the brain caused by mutation of the Notch3 gene. Very often, this disease is misdiagnosed. We examined skin biopsies in two members of the first discovered Serbian family affected by CADASIL. Electron microscopy showed that skin blood vessels of both patients contain numerous deposits of granular osmiophilic material (GOM) around vascular smooth muscle cells (VSMCs). We observed degeneration of VSMCs, reorganization of their cytoskeleton and dense bodies, disruption of myoendothelial contacts, and apoptosis. Our results suggest that the presence of GOM in small skin arteries represents a specific marker in diagnosis of CADASIL.

scholarly journals Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy

2011 ◽  
Vol 68 (5) ◽  
pp. 455-459 ◽  
Author(s):  
Zeljko Krsmanovic ◽  
Evica Dincic ◽  
Smiljana Kostic ◽  
Vesna Lackovic ◽  
Milos Bajcetic ◽  
...  
Keyword(s):  
Autosomal Dominant ◽  
Case Reports ◽  
Pathological Process ◽  
Unknown Etiology ◽  
Cognitive Deterioration ◽  
Distinctive Pattern ◽  
Ultrastructural Examination ◽  
Notch 3 ◽  
Skin Biopsies ◽  
The Brain

Introduction. Fast and precise diagnostics of the disease from the large group of adult leukoencephalopathy is difficult but responsible job, because the outcome of the disease is very often determined by its name. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is caused by the mutation of Notch 3 gene on chromosome locus 19p13. Beside the brain arterioles being the main disease targets, extracerebral small blood vessels are affected by the pathological process. Clinically present signs are recurrent ischemic strokes and vascular dementia. CADASIL in its progressive form shows a distinctive pattern of pathological changes on MRI of endocranium. The diagnosis is confirmed by the presence of granular osmiophilic material (GOM) in histopathological skin biopsies. Case reports. Two young adult patients manifested ischemic strokes of unknown etiology, cognitive deterioration, migraine and psychopathological phenomenology. MRI of endocranium pointed on CADASIL. Ultrastructural examination of skin biopsy proved the presence of GOM in the basal lamina and near smooth muscle cells of arteriole dermis leading to CADASIL diagnosis. The presence of GOM in histopathological preparation is 100% specific for CADASIL. The patients were not searched for mutation in Notch 3 gene on chromosome 19, because some other leukoencephalopathy was disregarded. Conclusion. Suggestive clinical picture, distinctive finding of endocranium MRI, the presence of GOM by ultrastructural examination of histopathological skin biopsies are sufficient to confirm CADASIL diagnosis.

The Neurovascular Unit: Focus on the Regulation of Arterial Smooth Muscle Cells

2020 ◽  
Vol 16 (5) ◽  
pp. 502-515 ◽  
Author(s):  
Patrícia Quelhas ◽  
Graça Baltazar ◽  
Elisa Cairrao
Keyword(s):  
Blood Flow ◽  
Smooth Muscle ◽  
Smooth Muscle Cells ◽  
Blood Vessels ◽  
Neurovascular Unit ◽  
Muscle Cells ◽  
Cerebral Blood Vessels ◽  
Mural Cells ◽  
Brain Pericytes ◽  
The Brain

The neurovascular unit is a physiological unit present in the brain, which is constituted by elements of the nervous system (neurons and astrocytes) and the vascular system (endothelial and mural cells). This unit is responsible for the homeostasis and regulation of cerebral blood flow. There are two major types of mural cells in the brain, pericytes and smooth muscle cells. At the arterial level, smooth muscle cells are the main components that wrap around the outside of cerebral blood vessels and the major contributors to basal tone maintenance, blood pressure and blood flow distribution. They present several mechanisms by which they regulate both vasodilation and vasoconstriction of cerebral blood vessels and their regulation becomes even more important in situations of injury or pathology. In this review, we discuss the main regulatory mechanisms of brain smooth muscle cells and their contributions to the correct brain homeostasis.

scholarly journals FILAMENT ULTRASTRUCTURE AND ORGANIZATION IN VERTEBRATE SMOOTH MUSCLE

1967 ◽  
Vol 35 (2) ◽  
pp. 303-321 ◽  
Author(s):  
Bernard J. Panner ◽  
Carl R. Honig
Keyword(s):  
Electron Microscopy ◽  
Skeletal Muscle ◽  
Smooth Muscle ◽  
Actin Filaments ◽  
Heavy Meromyosin ◽  
Dense Bodies ◽  
Contraction Mechanism ◽  
Dark Staining ◽  
Technical Factors ◽  
Low Ionic Strength

Using a variety of preparative techniques for electron microscopy, we have obtained evidence for the disposition of actin and myosin in vertebrate smooth muscle. All longitudinal myofilaments seen in sections appear to be actin. Previous reports of two types of longitudinal filaments in sections are accounted for by technical factors, and by differentiated areas of opacity along individual filaments. Dense bodies with actin emerging from both ends have been identified in homogenates, and resemble Z discs from skeletal muscle (Huxley, 1963). In sections, short, dark-staining lateral filaments 15–25 A in diameter link adjacent actin filaments within dense bodies and in membrane dense pataches. They appear homologous with Z-disc filaments. Similar lateral filaments connect actin to plasma membrane. Dense bodies and dense patches, therefore, are attachment points and denote units analogous to sarcomeres. In glycerinated, methacrylate-embedded sections, lateral processes different in length and staining characteristics from lateral filaments in dense bodies exist at intervals along actin filaments. These processes are about 30 A wide and resemble heavy meromyosin from skeletal muscle. They also resemble heads of whole molecules of myosin in negatively stained material from gizzard homogenates. Intact single myosin molecules and dimers have been found, both free and attached to actin, even in media of very low ionic strength. Myosin can, therefore, exist in relatively disaggregated form. Models of the contraction mechanism of smooth muscle are proposed. The unique features are: (1) Myosin exists as small functional units. (2) Movement occurs by interdigitation and sliding of actin filaments.

Studies of the innervation of rabbit myometrium and cervix

1989 ◽  
Vol 67 (8) ◽  
pp. 837-844 ◽  
Author(s):  
R. Bulat ◽  
M. S. Kannan ◽  
R. E. Garfield
Keyword(s):  
Electron Microscopy ◽  
Smooth Muscle ◽  
Blood Vessels ◽  
Spontaneous Activity ◽  
Isometric Contraction ◽  
Glyoxylic Acid ◽  
Adrenergic Innervation ◽  
Significant Inhibition ◽  
Adrenergic Nerves ◽  
Field Stimulation

We characterized the innervation of isolated circular and longitudinal-oriented muscle strips from the nulliparous rabbit uterus and cervix by field stimulation (FS). FS with increasing frequency (2.5–50 pps) and voltage (2.5–70 V) caused graded increases in isometric contraction with no relaxation or inhibition of spontaneous activity. Tetrodotoxin(TTX, 3.1 × 10−6 M) significantly reduced the FS response by 75% in all strips at higher stimulus frequencies. Contractile responses to FS were also significantly inhibited by atropine (3.5 × 10−6 M) in circular uterus and in longitudinal cervix. Guanethidine (5 × 10−6 M) reduced the response in all strips, as did phentolamine (3.6 × 10−6 M) in longitudinal uterus and circular cervix. Propranolol (3.9 × 10−6 M) did not significantly change the response in longitudinal uterus or circular cervix. In longitudinal uterus, combined guanethidine and atropine produced significant inhibition, but not statistically different from either drug alone. Similar results were seen in circular uterus. Electron microscopy and glyoxylic acid histofluorescence indicate that both blood vessels and smooth muscle in rabbit uterus are supplied with adrenergic nerves. The results suggest the presence of TTX-sensitive adrenergic and cholinergic excitatory innervation of rabbit uterus and cervix.Key words: uterus, myometrium, cervix, adrenergic innervation.

scholarly journals Intermediate-sized filaments of the prekeratin type in myoepithelial cells.

1980 ◽  
Vol 84 (3) ◽  
pp. 633-654 ◽  
Author(s):  
W W Franke ◽  
E Schmid ◽  
C Freudenstein ◽  
B Appelhans ◽  
M Osborn ◽  
...  
Keyword(s):  
Electron Microscopy ◽  
Smooth Muscle ◽  
Smooth Muscle Cells ◽  
Immunofluorescence Microscopy ◽  
Myogenic Differentiation ◽  
Muscle Cells ◽  
Myoepithelial Cells ◽  
Sweat Glands ◽  
Dense Bodies ◽  
Cell Specialization

Myoepithelial cells from mammary glands, the modified sweat glands of bovine muzzle, and salivary glands have been studied by electron microscopy and by immunofluorescence microscopy in frozen sections in an attempt to further characterize the type of intermediate-sized filaments present in these cells. Electron microscopy has shown that all myoepithelial cells contain extensive meshworks of intermediate-sized (7--11-nm) filaments, many of which are anchored at typical desmosomes or hemidesmosomes. The intermediate-sized filaments are also intimately associated with masses of contractile elements, identified as bundles of typical 5--6-nm microfilaments and with characteristically spaced dense bodies. This organization resembles that described for various smooth muscle cells. In immunofluorescence microscopy, using antibodies specific for the various classes of intermediate-sized filaments, the myoepithelial cells are strongly decorated by antibodies to prekeratin. They are not specifically stained by antibodies to vimentin, which stain mesenchymal cells, nor by antibodies to chick gizzard desmin, which decorate fibrils in smooth muscle Z bands and intercalated disks in skeletal and cardiac muscle of mammals. Myoepithelial cells are also strongly stained by antibodies to actin. The observations show (a) that the epithelial character, as indicated by the presence of intermediate-sized filaments of the prekeratin type, is maintained in the differentiated contractile myoepithelial cell, and (b) that desmin and desmin-containing filaments are not generally associated with musclelike cell specialization for contraction but are specific to myogenic differentiation. The data also suggest that in myoepithelial cells prekeratin filaments are arranged--and might function--in a manner similar to the desmin filaments in smooth muscle cells.

Electron microscopy of the glio-vascular organization of the brain of octopus

1969 ◽  
Vol 255 (797) ◽  
pp. 13-32 ◽  
Keyword(s):  
Electron Microscopy ◽  
Smooth Muscle ◽  
Blood Vessels ◽  
Light And Electron Microscopy ◽  
Muscle Cells ◽  
Medium Size ◽  
Muscle Fibres ◽  
Special Importance ◽  
Form Complex ◽  
Large Neurons

The glio-vascular organization of the octopus brain has been studied by light and electron microscopy. The structure of the walls of the blood vessels has been described. Two types of neuroglia can be recognized, the fibrous and protoplasmic glia; also enigmatic dark cells. Most blood vessels in the neuropil are surrounded by extracellular zones containing collagen. These zones give off glio-vascular tunnels (strands) that penetrate the neuropil in a complex network. The extracellular zones and tunnels contain in addition to collagen, smooth muscle cells and fibrocytes. Glial processes surround the extracellular zones and incompletely partition them from the neuropil. The small neuronal perikarya have no glial folds around them. The medium-size cells have thin glial sheets or finger processes related to their surfaces, which may indent the cells to form small trophospongia. The large neurons of the suboesophageal lobe have complex glial sheaths interspersed with extracellular channels. Both penetrate the neurons to form complex trophospongia. A new form of extracellular material has been observed in these extracellular channels. The occurrence of trophospongia in vertebrate and invertebrate neurons may be correlated with the absence of dendrites. Special problems discussed include the nature of the trophospongial function, the question of fluid-filled extracellular zones and their possible function as lymph channels, and the presence in some of them of haemocyanin molecules identical with those in the blood vessels. Perhaps of special importance is the observation that the lobes of the octopus brain are permeated with extracellular tunnels containing smooth muscle fibres, but it still needs to be determined whether or not the muscle cells in the tunnels of the neuropil actively contract and massage the neuropil to facilitate metabolic and other exchanges.

Glomangiomyoma (Glomus Tumor) of the Kidney

2005 ◽  
Vol 129 (9) ◽  
pp. 1172-1174 ◽  
Author(s):  
Noman H. Siddiqui ◽  
Agnieszka Rogalska ◽  
Indu S. Basil
Keyword(s):  
Electron Microscopy ◽  
Smooth Muscle ◽  
Glomus Tumor ◽  
Smooth Muscle Actin ◽  
Muscle Actin ◽  
Thin Filaments ◽  
Glomus Tumors ◽  
Computed Tomographic ◽  
Dense Bodies ◽  
Computed Tomographic Scan

Abstract We report herein a case of glomus tumor arising in the kidney of a 55-year-old woman, which was found incidentally on a computed tomographic scan. Partial nephrectomy revealed a 2-cm encapsulated mass that was architecturally similar to glomus tumor. Immunohistochemistry showed positivity of tumor cells for vimentin and smooth muscle actin. On electron microscopy, cytoplasmic thin filaments and dense bodies were seen, confirming the smooth muscle nature of the tumor. Glomus tumors arising in visceral organs are rare, and those arising in kidney are exceedingly rare.

scholarly journals A RARE CASE OF PSEUDOXANTHOMA ELASTICUM

2020 ◽  
pp. 1-1
Author(s):  
Sucheta Pathania
Keyword(s):  
Connective Tissue ◽  
Effective Treatment ◽  
Blood Vessels ◽  
Rare Case ◽  
Autosomal Dominant ◽  
Pseudoxanthoma Elasticum ◽  
Skin Lesions ◽  
Elastic Tissue ◽  
Connective Tissue Disorders ◽  
Skin Blood Vessels

Pseudoxanthoma elasticum (PXE) is also known as Gronblad-Strandberg syndrome. It belongs to the group of connective tissue disorders that affect the elastic tissue of the skin, blood vessels, and the eyes. Both autosomal dominant and recessive patterns of inheritance can be seen. Skin lesions consist of small, yellowish papules in rows or a lacy pattern, which may coalesce to form larger patches. The skin is soft, lax and slightly wrinkled. Common sites affected in PXE are the sides of the neck, below the collar bones, the armpits, abdomen, groins, perineum and thighs. Currently there is no effective treatment for the condition. As it can be passed to next generation genetic counselling can be helpful.

Anatomical relationships of the pia mater to cerebral blood vessels in man

1986 ◽  
Vol 65 (3) ◽  
pp. 316-325 ◽  
Author(s):  
Margaret Hutchings ◽  
Roy O. Weller
Keyword(s):  
Electron Microscopy ◽  
Cerebral Cortex ◽  
Blood Vessels ◽  
Subarachnoid Space ◽  
Blood Cells ◽  
Inflammatory Cells ◽  
Perivascular Spaces ◽  
Pia Mater ◽  
Transmission Electron ◽  
The Brain

✓ Using scanning and transmission electron microscopy and light microscopy, the authors studied the human pia mater and its relationship to the entry of blood vessels into the normal cerebral cortex. The purpose of this investigation was to examine the long-established concept that the subarachnoid space communicates directly with the perivascular spaces of the cerebral cortex. Brains obtained post mortem from subjects with recent subarachnoid hemorrhage (SAH) and purulent leptomeningitis were studied by light microscopy to determine the permeability of the pia mater to red blood cells and inflammatory cells. Scanning electron microscopy showed that the normal pia mater is a flat sheet of cells that is reflected from the surface of the brain to form the outer coating of the meningeal vessels in the subarachnoid space. Transmission electron microscopy confirmed that the cells of the pia mater are joined by junctional complexes and form a continuous sheet that separates the subarachnoid space on one side from the subpial and perivascular spaces on the other. Thus, neither the pia mater nor the subarachnoid space extends into the brain beside blood vessels as they enter the cerebral cortex. The perivascular spaces were, in fact, found to be confluent with the subpial space and not with the subarachnoid space. In cases of recent SAH, red blood cells did not enter the perivascular spaces from the subarachnoid space; neither did India ink injected post mortem into the subarachnoid space pass into the perivascular spaces. The results of these crude tracer studies suggest that the pia mater is an effective barrier to the passage of particulate matter. Histological examination of brains of patients who had died with purulent leptomeningitis showed that inflammatory cells were present in the cortical perivascular spaces and in the contiguous subpial spaces. The presence of a large number of inflammatory cells in the subarachnoid space suggests that inflammatory cells readily penetrate the pia mater that separates the perivascular spaces from the subarachnoid space. The permeability of the pia mater to small molecular weight substances is briefly discussed.

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