ABSTRACT: A 44-year-old man presented with oculobulbar weakness approximately 5 years after autologous bone marrow transplantation (BMT). His workup led to the diagnosis of muscle-specific kinase-antibody-related myasthenia gravis (MG). There has been only one case report of muscle-specific kinase-antibody-positive MG after BMT, which was allogeneic. We report the first case ...

In this study we describe a case of a term infant with the neurological variant of Waardenburg syndrome type 4 (i.e., PCWH = peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, Waardenburg syndrome, and Hirschsprung disease, as defined in OMIM #609136) due to a novel heterozygous base exchange (c.671C>G) in exon 4 ...

We report a 45-year-old man with myositis associated with clonal expansion of γδ T cells. He was referred to our hospital because of slowly progressive (over 10 years) muscle weakness. On neurological examination, weakness and muscle atrophy were noted in the proximal upper and lower limbs. The level of creatinine ...

Skeletal myogenesis is extensively influenced by the surrounding environment. However, how the extracellular matrix (ECM) affects morphogenesis of muscles is not well understood. We mapped the three-dimensional (3D) organization of fibronectin, tenascin, and laminin by immunofluorescence during early epaxial myogenesis in mouse embryos. We define four stages of dermomyotome/myotome development ...

New neurophysiological insights into the natural behaviour of dystonia, obtained during the successful botulinum toxin A (BoNT) treatment of the disorder, have urged the inclusion of sensory (and particularly somatosensory) mechanisms into the pathophysiological background of dystonia. Muscle spindles play a pivotal role in the generation of dystonic movements. Abnormal ...

The unique ability to stimulate bilaterally, extracranially, and non-invasively may represent a significant advantage to invasive neuromodulation therapies. In humans thus far the technique has been applied noninvasively, and is termed external trigeminal nerve stimulation (eTNSTM).

Genetic management of Mexican gray wolves includes semen banking, but due to the small number of animals in the population and handling restrictions, improvements in semen collection and cryopreservation rely on results from studies of domestic dogs. Semen collection from wolves requires anesthesia and electroejaculation, which introduce potentially important variables ...

Aim: Scaffold with micro-channels has shown great promise in facilitating axonal regeneration after peripheral nerve injury. Significant research has focused on mimicking, in terms of composition and function, the ability of the basement membrane of Schwann cells to both promote and guide axonal regeneration. We aim to investigate the ability ...

Administration after spinal cord injury (SCI) of methylprednisolone (MP) for 24-48 h has been suggested to improve functional outcome. The safety of this approach has been questioned because of the known adverse effects of glucocorticoids on skeletal muscle and the immune system. The purpose of this study was to explicitly ...

Heat stress is one of the hypertrophic stimuli on mammalian skeletal muscle. Nuclear factor-κB (NF-κB) signaling plays an important role in the regulation of skeletal muscle mass. However, the effects of heat stress on NF-κB signaling in skeletal muscle cells remain unclear. Effects of heat stress and/or administration of BAY11-7082, ...

The purpose of this study is to characterize the smooth muscle differentiation of purified human muscle-derived cells (hMDCs). The isolation and purification of hMDCs were conducted by modified preplate technique and Dynal CD34 cell selection. Smooth muscle cell differentiation was induced by the use of smooth muscle induction medium (SMIM) ...

Metronomic chemotherapy with alkylating agents has been shown to suppress tumor angiogenesis and prevent tumor recurrence in some settings. The use of adjuvant lomustine (1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea) administered in a metronomic fashion has not been evaluated in dogs. Oral metronomic administration of lomustine will be well tolerated in dogs with spontaneously occurring ...

Leiomyomas of the ventral ligament (LVLs) of the oviduct from 2-year-old spent layers were examined. These tumours can be present either as single large masses or as multiple smaller nodules. The most common site of origin of the tumours was the centre of the free margin of the ventral ligament, ...

Age-related increases in oxidative stress contribute to impaired skeletal muscle vascular control. However, recent evidence indicates that antioxidant treatment with tempol (4-hydroxy-2,2,6,6-tetramethylpiperidine-1-oxyl) attenuates flow-mediated vasodilation in isolated arterioles from the highly oxidative soleus muscle of aged rats. Whether antioxidant treatment with tempol evokes similar responses in vivo at rest and ...

The differentiation of adipose-derived stem cells (ASCs) into functional smooth muscle cells has received limited investigation. Various methodologies for both in vitro and in vivo differentiation is described. In vitro differentiation is obtained by either chemical or mechanical stimulation, and is determined by expression of smooth muscle cell markers. In ...

Stricture formation occurs in ≈30% of patients with Crohn's disease (CD) and is a significant cause of morbidity. Strictures are characterized by intestinal smooth muscle cell hyperplasia, smooth muscle cell hypertrophy, and fibrosis due to excess net extracellular matrix production, including collagen. Transforming growth factor-β1 (TGF-β1) has profibrotic effects in ...

Cardiovascular disease is one of the leading causes of death in the United States, and new treatments need to be developed in order to provide novel therapies. Tissue engineering aims to develop biologic substitutes that restore tissue function. The purpose of the current study was to construct cell-based pumps, which ...

Aim:  At the interface of tissue and capillaries, pericytes (PC) may generate electrical signals to be conducted along the skeletal muscle vascular network, but they are functionally not well characterized. We aimed to isolate and cultivate muscle PC allowing to analyse functional properties considered important for signal generation and conduction. ...

An adult male owl monkey (Aotus nancymae) underwent a splenectomy. When the spleen was removed, a small, nodular mass slightly bulging over the splenic surface was noted. The mass was examined by light and transmission electron microscopy and by immunohistochemistry. On light microscopy, the mass was well-circumscribed, non-encapsulated, and composed ...

The enteric nervous system (ENS) continues its structural and functional growth after birth, with formation of ganglia and the innervation of growing smooth muscle. However, little is known about factors in the postnatal intestine that influence these processes. We examined the presence and potential role of glial cell line-derived nerve ...

To determine whether S-nitrosylation of connexins (Cxs) modulates gap junction communication between endothelium and smooth muscle. Heterocellular communication is essential for endothelium control of smooth muscle constriction; however, the exact mechanism governing this action remains unknown. Cxs and NO have been implicated in regulating heterocellular communication in the vessel wall. ...

Facioscapulohumeral muscular dystrophy (FSHD) is an inherited disease, and although strongly suggested, a contribution of inflammation to its pathogenesis has never been demonstrated. In FSHD patients, we found by immunohistochemistry inflammatory infiltrates mainly composed by CD8(+) T cells in muscles showing hyperintensity features on T2-weighted short tau inversion recovery magnetic ...

The ductus arteriosus is an arterial vessel that shunts blood flow away from the lungs during fetal life, but normally occludes after birth to establish the adult circulation pattern. Failure of the ductus arteriosus to close after birth is termed patent ductus arteriosus and is one of the most common ...

Polymer scaffolds that direct elongation and orientation of cultured cells can enable tissue engineered muscle to act as a mechanically functional unit. We combined micromolding and microablation technologies to create muscle tissue engineering scaffolds from the biodegradable elastomer poly(glycerol sebacate). These scaffolds exhibited well defined surface patterns and pores and ...

In chick and mouse embryogenesis, a population of cells described as the secondary heart field (SHF) adds both myocardium and smooth muscle to the developing cardiac outflow tract (OFT). Following this addition, at approximately HH stage 22 in chick embryos, for example, the SHF can be identified architecturally by an ...

One major unresolved issue in muscle blood flow regulation is that of the role of circulating versus interstitial vasodilatory compounds. The present study determined adenosine-induced formation of NO and prostacyclin in the human muscle interstitium versus in femoral venous plasma to elucidate the interaction and importance of these vasodilators in ...

The proliferation and migration of vascular smooth muscle cells (SMCs) from the media toward the intimal layer are key components in vascular proliferative diseases. In addition, the differentiation of circulating bone marrow-derived mononuclear cells (BMMCs) into SMCs has been described to contribute to lesion progression in experimental models of atherosclerosis, ...

Structural organization of hepatic portal vein (HPV) was examined in adult rats by means of light and electron microscopy. Three characteristic features were found in the wall structure of rat HPV. (1) Tunica media consisted of two kinds of smooth muscle. The inner circular smooth muscle (CSM) was composed with ...

Interstitial cells of Cajal (ICCs) are located in most parts of the digestive system. Although they have been found over 100 years, their functions began to be unravelled only recently. ICCs are considered as pacemaker cells which elicit spontaneous rhythmic electric activity termed "basic electrical rhythm" or "slow waves" in ...

Multipotent Isl1(+) heart progenitors give rise to three major cardiovascular cell types: cardiac, smooth muscle, and endothelial cells, and play a pivotal role in lineage diversification during cardiogenesis. A critical question is pinpointing when this cardiac-vascular lineage decision is made, and how this plasticity serves to coordinate cardiac chamber and ...

Control of gastrointestinal (GI) movements by enteric motoneurons is critical for orderly processing of food, absorption of nutrients and elimination of wastes. Work over the past several years has suggested that motor neurotransmission is more complicated than simple release of transmitter from nerve terminals and binding of receptors on smooth ...

To determine if mast cell activation in skeletal muscle contributes to overload-induced angiogenesis. Extensor digitorum longus muscle was overloaded through extirpation of the synergist muscle tibialis anterior. Muscles were removed after 1, 2, 4, 7 or 14 days, and mast cell density and degranulation were quantified by histology. The mast ...

Both rat derived vascular smooth muscle cells (SMC) and human myofibroblasts contain α smooth muscle actin (SMA), but they utilize different mechanisms to contract populated collagen lattices (PCLs). The difference is in how the cells generate the force that contracts the lattices. Human dermal fibroblasts transform into myofibroblasts, expressing α-SMA ...

From the muscles that control the blink of your eye to those that allow you to walk, the basic architecture of muscle is the same: muscles consist of bundles of the unit muscle cell, the muscle fiber. The unique morphology of the individual muscle fiber is dictated by the functional ...

With the advent of genetically engineered mice, it seems important to develop a mouse model of cavernous nerve injury (CNI). To establish a mouse model of CNI induced either by nerve crushing or by neurectomy and to evaluate time-dependent derangements in penile hemodynamics in vivo and subsequent histologic alterations in ...

Doxazosin (DOX), an α-adrenoceptor antagonist, induces the relaxation of smooth muscle cell tonus and reduces the clinical symptoms of benign prostatic hyperplasia (BPH). However, the effects of DOX in the prostate stromal microenvironment are not fully known. In a previous study, we showed that DOX treatment for 30 days increased ...

The molecular regulation of smooth muscle cell (SMC) behavior is reviewed, with particular emphasis on stimuli that promote the contractile phenotype. SMCs can shift reversibly along a continuum from a quiescent, contractile phenotype to a synthetic phenotype, which is characterized by proliferation and extracellular matrix (ECM) synthesis. This phenotypic plasticity ...

The muscle layer in the cystic duct and common bile duct is not well defined, and it is unresolved whether it represents muscularis mucosae or muscularis propria. Smoothelin is a novel smooth muscle-specific contractile protein expressed only in fully differentiated smooth muscle cells of the muscularis propria and not in ...

Cardiovascular diseases represent the most common cause of death in industrialized countries. In this context vascular smooth muscle cells (SMCs) are a major key player that is involved in pathological processes like hypertension and atherosclerosis. Therefore the pharmaceutical industry is intensively investigated in developing non-destructive and label-free monitoring techniques for ...

Different muscles in a beef carcass are known to respond differently to the same stimulus or animal growth pattern or both. This may complicate the search by the meat industry for production methods to render meat tender. One of the major differences between muscles in the same carcass is in ...

Nippostrongylus brasiliensis infections generate pulmonary pathologies that can be associated with strong T(H)2 polarization of the host's immune response. We present data demonstrating N. brasiliensis-driven airway mucus production to be dependent on smooth muscle cell interleukin 4 receptor-α (IL-4Rα) responsiveness. At days 7 and 10 post infection (PI), significant airway ...

Serum response factor (SRF) is a widely expressed protein that plays a key role in the regulation of smooth muscle differentiation, proliferation, migration, and apoptosis. It is generally accepted that one mechanism by which SRF regulates these diverse functions is through pathway-specific cofactor interactions. A novel SRF cofactor, chromodomain helicase ...

The purpose of our study was to investigate the role of endogenous p63RhoGEF in G(q/11)-dependent RhoA activation and signaling in rat aortic smooth muscle cells (RASMCs). Therefore, we studied the expression and subcellular localization in freshly isolated RASMCs and performed loss of function experiments to analyze its contribution to RhoGTPase ...

The wall of hollow organs of vertebrates is a unique structure able to generate active tension and maintain a nearly constant passive stiffness over a large volume range. These properties are predominantly attributable to the smooth muscle cells that line the organ wall. Although smooth muscle is known to possess ...

Ischemic wounds are characterized by oxygen levels lower than that of healthy skin (hypoxia) and poor healing. To better understand the pathophysiology of impaired wound healing, we investigated how switching from high (21%) to low (2%) oxygen levels directly affects cultured skin myofibroblasts, essential cells for the normal wound repair ...

Recently, overexpression of the genes TMEM16A and TMEM16B has been shown to produce currents qualitatively similar to native Ca(2+)-activated Cl(-) currents (I(ClCa)) in vascular smooth muscle. However, there is no information about this new gene family in vascular smooth muscle, where Cl(-) channels are a major depolarizing mechanism. Qualitatively similar ...

Angiomyolipomas (AMLs) are benign tumours characterized by fat, smooth muscle and vascular components. Epithelioid AML is a recognized variant of AML that is comprised of epithelioid smooth muscle cells. We present a case of a 41-year-old male who presented with light-headedness, dizziness, right-sided abdominal pain and, on subsequent computed tomography, ...

Purpose: The purpose of this study was to determine whether periurethral injection of allogenic mesenchymal stem cells (MSCs) could increase the leak point pressure (LPP) in a rat model of stress urinary incontinence. Materials and Methods: Female Sprague-Dawley rats (230-240 g, n = 30) were divided into 3 groups: sham ...

Protease-activated receptors (PARs) are expressed on structural and immune cells. Control of initiation, duration, and magnitude of PAR effects is linked to the level of receptor expression, availability of proteases, and the intracellular signal transduction machinery. We investigated nematode infection-induced changes in PAR(2) expression and the impact on smooth muscle ...

Angiomatoid lesions in a lymph node associated with a thyroid carcinoma of a dog were restricted to the subcapsular and medullary sinuses. Lymphoid atrophy was present, but nodal architecture was not distorted and normal structures were not invaded. Immunohistochemical staining indicated that the vascular spaces formed by spindloid cells were ...