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muscle spindle - Top 30 Publications

Functional analysis of human intrafusal fiber innervation by human γ-motoneurons.

Investigation of neuromuscular deficits and diseases such as SMA, as well as for next generation prosthetics, utilizing in vitro phenotypic models would benefit from the development of a functional neuromuscular reflex arc. The neuromuscular reflex arc is the system that integrates the proprioceptive information for muscle length and activity (sensory afferent), to modify motoneuron output to achieve graded muscle contraction (actuation efferent). The sensory portion of the arc is composed of proprioceptive sensory neurons and the muscle spindle, which is embedded in the muscle tissue and composed of intrafusal fibers. The gamma motoneurons (γ-MNs) that innervate these fibers regulate the intrafusal fiber's stretch so that they retain proper tension and sensitivity during muscle contraction or relaxation. This mechanism is in place to maintain the sensitivity of proprioception during dynamic muscle activity and to prevent muscular damage. In this study, a co-culture system was developed for innervation of intrafusal fibers by human γ-MNs and demonstrated by morphological and immunocytochemical analysis, then validated by functional electrophysiological evaluation. This human-based fusimotor model and its incorporation into the reflex arc allows for a more accurate recapitulation of neuromuscular function for applications in disease investigations, drug discovery, prosthetic design and neuropathic pain investigations.

Distributed force feedback in the spinal cord and the regulation of limb mechanics.

This review is an update on the role of force feedback from Golgi tendon organs in the regulation of limb mechanics during voluntary movement. Some current ideas about the role of force feedback are based on circuit motifs linking idealized systems of agonists, synergists and antagonistic muscles. Force feedback is widely distributed across the muscles of a limb and cannot be understood based on these circuit motifs. Muscle architecture similarly cannot be understood in terms of idealized systems. It is hypothesized that distributed force feedback better represents the complex mechanical interactions of muscles, including viscoelastic coupling and inertial coupling across joints and axes of rotation. Signals from Golgi tendon organs appear to represent the stresses in the musculosketal network born by muscle articulations, myofascial force transmission and inertial coupling. Together with the strains of muscle fascicles measured by length feedback from muscle spindle receptors, this integrated proprioceptive feedback represents the mechanical state of the musculoskeletal system. Within the spinal cord, force feedback has excitatory and inhibitory components that co-exist in various combinations based on motor task and integrated with length feedback at the pre-motoneuronal and motoneuronal levels. It is concluded that, in agreement with other investigators, that autogenic, excitatory force feedback contributes to propulsion and weight support. It is further concluded that coexistent inhibitory force feedback, together with length feedback, evolved to manage interjoint coordination in the face of destabilizing inertial forces and positive force feedback, as required by the accelerations and changing directions of both predator and prey.

Intradermal Proliferative Fasciitis Occurring With Chondrodermatitis Nodularis Helicis.

Nodular fasciitis is a benign myofibroblastic tumor. Its uncommon variant, proliferative fasciitis (PF), can present in an even less common intradermal form. We report a case of intradermal PF of the ear in a 45-year-old man who presented with recurrent episodes of pain and swelling of the lesion. Histologic examination showed a dermal, nodular proliferation of ganglion-like basophilic fibroblasts with prominent nuclei and nucleoli, admixed with foamy histiocytes and areas of spindle cells arranged in intersecting fascicles in a fibromyxoid background. Lesional cells stained positive for smooth muscle actin and were negative for AE1/AE3, p63, and Melan-A. CD68 highlighted intervening histiocytes. We postulate that the underlying chondrodermatitis nodularis helicis was a triggering etiology, consistent with the current speculation that intradermal PF results from trauma.

Therapeutic effects of conditioned medium from bone marrow-derived mesenchymal stem cells on epithelial-mesenchymal transition in A549 cells.

Pulmonary fibrosis (PF) is a chronic lung disease. The transforming growth factor-β1 (TGF-β1)/Smad3 signaling pathway plays an important role in the pathogenesis of pulmonary fibrosis. Bone marrow-derived mesenchymal stem cells (BMSCs) have been shown to be a modulator of the molecular aspects of the fibrosis pathway. However, it is still unknown as to whether the conditioned medium from BMSCs (BMSCs-CM) inhibits the epithelial-mesenchymal transition (EMT) process. This study confirmed the hypothesis that BMSCs-CM exerts an anti-fibrotic effect on human type II alveolar epithelial cells (A549) by suppressing the phosphorylation of Smad3. We used the A549 cells in vitro to detect morphological evidence of EMT by phase-contrast microscopy. These cells were randomly divided into 4 groups as follows: the control group, the TGF-β1 group, the SIS3 (specific inhibitor of Smad3) group and the BMSCs-CM group. The immunofluorescence method was used to determined the location of E-cadherin (E-calcium mucins; E-cad), α-smooth muscle actin (α-SMA) and p-Smad3. The expression levels of E-cad, CK8, α-SMA, vimentin, p-Smad3, Snail1, collagen I (COLI) and collagen III (COLIII) were detected by western blot analysis. Following exposure to TGF-β1, the A549 cells displayed a spindle-shaped fibroblast-like morphology. In accordance with these morphological changes, the expression levels of E-cad and CK8 were downregulated, while the expression levels of α-SMA and vimentin were upregulated. Along with this process, the expression levels of p-Smad3, Snail1, COLI and COLIII were increased. However, the cells in the BMSCs-CM group and SIS3 group exhibited a decrease in the levels of α-SMA and vimentin (which had been upregulated by TGF-β1), and an increase in the levels of E-cad and CK8 expression (which had been downregulated by TGF-β1). On the whole, these results indicated that BMSCs-CM suppressed the EMT which might be associated with TGF-β1/Smad3. This study provides the theoretical basis for the research of the mechanisms responsible for pulmonary disease.

Spindle associated membrane protein 1 (Samp1) is required for the differentiation of muscle cells.

Muscles are developed and regenerated in a differentiation process called myogenesis, which involves components of the nuclear envelope. We have investigated Samp1 (Spindle Associated Membrane Protein 1), a transmembrane nuclear envelope protein, which interacts with emerin and lamin A, both of which are linked to Emery-Dreifuss muscular dystrophy (EDMD). We found that the levels of Samp1 increased seven-fold during differentiation of mouse C2C12 muscle progenitor cells. To test if Samp1 could have a role in myogenesis we developed stable C2C12 knockdown cell lines expressing short hairpin RNA targeting Samp1 expression. The Samp1 depleted C2C12 cells displayed normal mobility and normal distribution of emerin and lamin A. However, Samp1 depletion increased ERK signaling and completely blocked differentiation of C2C12 cells, which failed to express myogenic marker proteins and failed to form myotubes. The block in myogenesis in Samp1 depleted cells was completely rescued by ectopic expression of RNAi resistant human Samp1, showing that Samp1 is required for muscle differentiation.

Unexpected pulmonary tumor: metastasis from a benign uterine leiomyoma in a post-menopausal woman: a case report.

The occurrence of lung metastasis from benign uterine leiomyomas is rarely reported especially in post menopausal women. The pathogenesis of these metastatic benign tumors still remains a subject of various speculations.

Quantitative input: output relationships between human soleus muscle spindle afferents and motoneurons.

A method is described which, for the first time, allows instantaneous estimation of the Ia fiber input to human soleus motoneurons following electrical stimulation of the tibial nerve. The basis of the method is to determine the thresholds of the most and least excitable 1a fibers to electrical stimulation, and to treat the intervening thresholds as having a normal distribution about the mean; the validity of this approach is discussed. It was found that, for the same Ia fibre input, the percentage of soleus motoneurons contributing to the H (Hoffmann)-reflex differed considerably among subjects; when the results were pooled, however, there was an approximately linear relationship between Ia input and motoneuron output. Weak extension of the great toe diminished the soleus motoneuron reflex discharge in all but 2 of 16 subjects; the results for weak ankle plantarflexion were less consistent but overall there was a reduction in soleus motoneuron output also. The methodology should provide new insights into disorders of movement and tone, especially as it permits estimates of motoneuron depolarization to be made.

The neural response properties and cortical organization of a rapidly adapting muscle sensory group response that overlaps with the frequencies that elicit the kinesthetic illusion.

Kinesthesia is the sense of limb movement. It is fundamental to efficient motor control, yet its neurophysiological components remain poorly understood. The contributions of primary muscle spindles and cutaneous afferents to the kinesthetic sense have been well studied; however, potential contributions from muscle sensory group responses that are different than the muscle spindles have not been ruled out. Electrophysiological recordings in peripheral nerves and brains of male Sprague Dawley rats with a degloved forelimb preparation provide evidence of a rapidly adapting muscle sensory group response that overlaps with vibratory inputs known to generate illusionary perceptions of limb movement in humans (kinesthetic illusion). This group was characteristically distinct from type Ia muscle spindle fibers, the receptor historically attributed to limb movement sensation, suggesting that type Ia muscle spindle fibers may not be the sole carrier of kinesthetic information. The sensory-neural structure of muscles is complex and there are a number of possible sources for this response group; with Golgi tendon organs being the most likely candidate. The rapidly adapting muscle sensory group response projected to proprioceptive brain regions, the rodent homolog of cortical area 3a and the second somatosensory area (S2), with similar adaption and frequency response profiles between the brain and peripheral nerves. Their representational organization was muscle-specific (myocentric) and magnified for proximal and multi-articulate limb joints. Projection to proprioceptive brain areas, myocentric representational magnification of muscles prone to movement error, overlap with illusionary vibrational input, and resonant frequencies of volitional motor unit contraction suggest that this group response may be involved with limb movement processing.

Transverse colon schwannoma treated by endoscopic mucosal resection: A case report.

Gastrointestinal (GI) schwannoma is one of the rarest tumors of the GI tract. We herein describe the case of a 70-year-old female patient who presented for surveillance colonoscopy. The examination detected a 1-cm polyp that was removed by snare cautery polypectomy. Immune and histochemical staining revealed spindle cells that were positive for S-100 and vimentin, but negative for CD34 and smooth muscle actin, consistent with GI schwannoma. This case is noteworthy as GI schwannomas usually present in the stomach, making the finding of a colonic schwannoma of clinical interest. Furthermore, the present case was treated by removing the tumor endoscopically, in contrast to more invasive methods.

Growth of doxorubicin-resistant undifferentiated spindle-cell sarcoma PDOX is arrested by metabolic targeting with recombinant methioninase.

Undifferentiated spindle-cell sarcoma (USCS) is a recalcitrant -cancer in need of individualized therapy. A high-grade USCS from a striated muscle of a patient was grown orthotopically in the right biceps femoris muscle of nude mice to establish a patient-derived orthotopic xenograft (PDOX) model. In a previous study, we evaluated the efficacy of standard first-line chemotherapy of doxorubicin (DOX), gemcitabine (GEM) combined with docetaxel (DOC), compared to pazopanib (PAZ), a multi-targeting tyrosine-kinase inhibitor, in an USCS PDOX model. In the present study, animal-bearing the USCS PDOX tumors were randomized into the following groups when tumor volume reached 100 mm3 : G1, untreated control without treatment; G2, DOX (3 mg/kg, intraperitoneal (i.p.) injection, weekly, for 2 weeks); G3, L-methionine α-deamino-γ-mercaptomethane lyase (recombinant methioninase [rMETase]) (100 unit/mouse, i.p., daily, for 2 weeks). Tumor sizes and body weight were measured with calipers and digital balance twice a week and methionine level of supernatants derived from sonicated tumors were measured. rMETase inhibited tumor growth compared to untreated controls and the DOX treated group on day 14 after initiation of treatment: control (G1): 347.6 ± 88.2 mm3 ; DOX (G2): 329.5 ± 78.8 mm3 , p = 0.670; rMETase (G3): 162.6 ± 51.2 mm3 , p = 0.0003. The mouse body weight of the treated mice was not significantly different from the untreated controls. Tumor L-methionine levels were reduced after the rMETase-treatment compared to untreated control and pre-rMETase treatment. We previously reported efficacy of rMETase against Ewing's sarcoma in a PDOX model. These studies suggest clinical development of rMETase, especially in recalcitrant cancers such as sarcoma. This article is protected by copyright. All rights reserved.

Primary leiomyosarcoma of the bone: a case report and a review of the literature.

Leiomyosarcoma (LMS) is a malignant sarcoma that can occur in different anatomic sites, including the bone, showing similar histological characteristics but heterogeneous clinical behavior and prognosis. Primary bone LMS was first described in 1965. It is a very rare sarcoma, accounting for <0.7% of all primary malignant bone tumors.

Clinicopathologic and molecular characteristics of malignant gastrointestinal neuroectodermal tumors.

Objective: To investigate the clinicopathologic and molecular characteristics, diagnostic, differential diagnostic and prognostic features of malignant gastrointestinal neuroectodermal tumor. Methods: Two cases of malignant gastrointestinal neuroectodermal tumor were retrieved; the clinical and radiologic features, histomorphology, immunophenotype, molecular genetics and prognosis were analyzed and the relevant literature reviewed. Results: Case 1 was a 57-year-old male, presented with recurrent abdominal pain and melena. Pelvic imaging showed a circumscribed thickening of the wall of a small intestinal segment, and a malignant lymphoma was favored. Case 2 was a 24-year-old male, presented with recurrent small intestinal malignancy. Imaging demonstrated multiple masses in the peritoneal and pelvic cavities, and a malignant gastrointestinal stromal tumor with multiple metastases was suspected. Grossly both tumors were located mainly in the muscularis propria of small intestine. Case 1 showed a single 5.5 cm tumor; and case 2 consisted of two tumors measuring 4 cm and 6 cm respectively. Microscopic examination of both tumors showed small round blue, but focally spindled or clear tumor cells in solid pattern. The tumor cells had scanty cytoplasm, indistinctive nucleoli and brisk mitoses. Osteoclast-like giant cells were dispersed within the stroma. In case 1 rosette-like and pseudo-papillary growth patterns were noted, and in case 2 there were variable-sized hemorrhagic cysts. By immunohistochemistry, both tumors showed strong and diffuse expression of SOX10 and S-100, and focal to diffuse expression of neuroendocrine markers (CD56 or synaptophysin). Case 2 exhibited focal reactivity to pan-cytokeratin. Both tumors lacked expression of markers associated with gastrointestinal stromal tumor, smooth muscle tumor, melanoma (HMB45 or Melan A), dendritic cell tumor and Ewing sarcoma. Fluorescence in situ hybridization analysis demonstrated EWSR1 rearrangement in both tumors and the next generation sequencing confirmed EWSR1-ATF1 gene fusion in case 2. At follow-up of 16 months, case 1 was recurrence or metastasis free; whereas case 2 showed multiple recurrences and metastases within 19 months although stable disease was transiently achieved when treated with combinations of multidrug and targeted chemotherapy. Conclusions: Malignant gastrointestinal neuroectodermal tumor is a rare and aggressive soft tissue sarcoma with a predilection for small intestine. It has distinctive morphologic, immunohistochemical and molecular characteristics and needs to be distinguished from other small blue round and spindle cell tumors that occur in the gut. Careful attentions to its characteristic histomorphology with the judicious use of immunohistochemistry and molecular genetics can help to distinguish this tumor from its many mimickers.

Myopericytoma of the neck originating in the middle scalene muscle: A case report.

We report a case of myopericytoma of the neck. A 23-year-old woman noticed a small, nontender mass in her left supraclavicular fossa. The mass had grown over a period of 5 months, prompting her to seek evaluation. On examination, no motor or sensory deficits were present. Imaging suggested that a mass had originated in the middle scalene muscle. Computed-tomography-guided core needle biopsy demonstrated a spindle-cell neoplasm with smooth-muscle differentiation. Complete surgical excision was performed. Histopathologic and immunohistochemical evaluations of the tissue sample suggested a myopericytoma. Myopericytoma is an extremely rare tumor of the head and neck. To the best of our knowledge, this is the first reported case of a myopericytoma originating in a scalene muscle.

Assisted reproductive technologies to prevent human mitochondrial disease transmission.

Mitochondria are essential cytoplasmic organelles that generate energy (ATP) by oxidative phosphorylation and mediate key cellular processes such as apoptosis. They are maternally inherited and in humans contain a 16,569-base-pair circular genome (mtDNA) encoding 37 genes required for oxidative phosphorylation. Mutations in mtDNA cause a range of pathologies, commonly affecting energy-demanding tissues such as muscle and brain. Because mitochondrial diseases are incurable, attention has focused on limiting the inheritance of pathogenic mtDNA by mitochondrial replacement therapy (MRT). MRT aims to avoid pathogenic mtDNA transmission between generations by maternal spindle transfer, pronuclear transfer or polar body transfer: all involve the transfer of nuclear DNA from an egg or zygote containing defective mitochondria to a corresponding egg or zygote with normal mitochondria. Here we review recent developments in animal and human models of MRT and the underlying biology. These have led to potential clinical applications; we identify challenges to their technical refinement.

The human platysma contains numerous muscle spindles.

The mimic muscles are usually described as containing no muscle spindles. In the present publication the human platysma was reinvestigated concerning its content of corpuscular sensors. Serial sections through the platysma of seven donors revealed numerous muscle spindles but no Pacini corpuscules. The muscle spindles were located in the cranial two-thirds of the platysma, and were evenly distributed with a tendency to have more spindles in the lateral part of the muscle. Immunohistochemical staining with S46 antibodies revealed a predominance of nuclear bag fibers. The results point to an extended function of the platysma as an afferent center of the lower face mimic muscles.

Modification of Masticatory Rhythmicity Leading to the Initiation of the Swallowing Reflex in Humans.

Modification of movements by proprioceptive feedback during mastication has an important role in shifting from the oral to the pharyngeal phase of swallowing. The aim of this study was to investigate the kinetics of masticatory muscles throughout a sequence of oropharyngeal swallowing and to present a hypothetical model of the involvement of the nervous system in the transition from mastication to the swallowing reflex. Surface electromyographic signals were recorded from the jaw-closing masseter muscles and the jaw-opening suprahyoid muscle group when a piece of bread (3-5 g) was ingested. Participants were not provided any additional instruction regarding how to chew and swallow. In the final stage of mastication, compared with other stages of mastication, the duration between sequential peak times of rhythmic activity of the masseter muscles was prolonged. Electromyography revealed no significant change in the suprahyoid muscle group. Accordingly, contraction of the jaw-closing muscles and the jaw-opening muscles altered from out-of-phase to in-phase. We have presented a hypothetical model based on the results of the present study, in which mastication shifts to the swallowing reflex when feed-forward inputs from rhythm generators for the jaw-closing and the jaw-opening muscles converge onto an assumed "convertor" neuron group concurrently. This model should contribute to understanding the pathophysiology of dysphagia.

Sarcoma Arising from the Chest Wall : A Case Report.

Chest wall contains a wide array of tissues ranging from soft tissues like skin and muscle to bone. A variety of sarcomas can present with a painful or painless mass, which often requires histological testing for diagnosis. Chest wall sarcomas are very rare entities which are often growing slow . A multidisciplinary team is necessary for the management of chest wall sarcomas. We present a case of a 30-year-old male with spindle cell sarcoma of the chest wall and he underwent wide local excision along with surgical reconstruction.

The relationship between the sensory responses to ankle-joint loading and corticomotor excitability.

Maintaining joint stability is dependent on the ability of the nervous system to sense and react to potentially injurious loads. In attempts to understand the neurophysiologic mechanisms underlying joint stability, this afferent and efferent activity has been quantified separately at the cortical, segmental and peripheral levels using various electrophysiologic techniques in vivo. However, no studies have attempted to quantify sensory and motor activation at multiple levels of the nervous system in a single subset, to understand potential adaptations for optimizing joint stability.

Positional Tremor and its Treatment.

Positional tremors arise when a patient's tremor is brought on during specific positioning of the involved body part. They can be distinguished from postural tremor, wherein a patient's tremor is elicited in any posture, and from task-specific tremor, wherein a patient's tremor occurs only during a certain task.

The resilient brain and the guardians of sleep: New perspectives on old assumptions.

Resilience is the capacity of a system, enterprise or a person to maintain its core purpose and integrity in the face of dramatically changed circumstances. In human physiology, resilience is the capacity of adaptively overcoming stress and adversity while maintaining normal psychological and physical functioning. In this review, we investigate the resilient strategies of sleep. First, we discuss the concept of brain resilience, highlighting the modular structure of small-world networking, neuronal plasticity and critical brain behavior. Second, we explore the contribution of sleep to brain resilience listing the putative factors that impair sleep quality and predict susceptibility to sleep disorders. The third part details the manifold mechanisms acting as guardians of sleep, i.e., homeostatic, circadian and ultradian processes, sleep microstructure (K-complexes, delta bursts, arousals, cyclic alternating pattern, spindles), gravity, muscle tone and dreams. Mapping and pooling together the guardians of sleep in a dynamic integrated framework might lead towards an objective measure of sleep resilience and identify effective personalized strategies (biological, pharmacological, behavioral) to restore or protect the core properties of healthy sleep.

Post-biopsy MRI changes in the size and enhancement of intramuscular myxomas: A report of two cases.

Intramuscular myxomas are benign soft-tissue tumors, characterized by bland spindle-shaped cells and fibroblasts within an abundant mucoid matrix on histologic examination. Classically, these are slowly enlarging masses which may occasionally cause pain, paresthesia, and muscle weakness secondary to mass effect. We present an interesting phenomenon of two histologically confirmed cases of intramuscular myxomas that exhibited size and enhancement changes on follow-up imaging after image-guided biopsy. To our knowledge, this is the first report to describe size and enhancement changes of intramuscular myxomas after biopsy.

Oxidized regenerated cellulose induces pleural thickening in patients with pneumothorax: possible involvement of the mesothelial-mesenchymal transition.

The pleural covering technique, i.e., wrapping a part of or the entire surface of the lung with oxidized regenerative cellulose (ORC), reinforces visceral pleura through pleural thickening for patients with pneumothorax and cystic lung diseases. However, it remains undetermined how ORC induces pleural thickening.

Inflammatory Myofibroblastic Tumor of the Breast Mimicking Malignancy in an Elderly Male.

Inflammatory myofibroblastic tumor (IMT) is a rare, distinctive lesion composed of a proliferation of myofibroblastic spindle cells accompanied by an inflammatory infiltrate. It was first described in the lung, but its occurrence at various extrapulmonary sites has also been reported. The literature mentions only a handful of cases of IMT in the breast and only 1 case in the male breast. We report the second case of IMT in the male breast.

Physiological tremor increases when skeletal muscle is shortened: implications for fusimotor control.

In tonic, isometric, plantarflexion contractions, physiological tremor increases as the ankle joint becomes plantarflexed. Modulation of physiological tremor as a function of muscle stretch differs from that of the stretch reflex amplitude. Amplitude of physiological tremor may be altered as a function of reflex pathway gains. Healthy humans likely increase their γ-static fusimotor drive when muscles shorten. Quantification of physiological tremor by manipulation of joint angle may be a useful experimental probe of afferent gains and/or the integrity of automatic fusimotor control.

Thalamo-insular pathway conveying orofacial muscle proprioception in the rat.

Little is known about how proprioceptive signals arising from muscles reach to higher brain regions such as the cerebral cortex. We have recently shown that a particular thalamic region, the caudo-ventromedial edge (VPMcvm) of ventral posteromedial thalamic nucleus (VPM), receives the proprioceptive signals from jaw-closing muscle spindles (JCMSs) in rats. In this study, we further addressed how the orofacial thalamic inputs from the JCMSs were transmitted from the thalamus (VPMcvm) to the cerebral cortex in rats. Injections of a retrograde and anterograde neuronal tracer, wheat-germ agglutinin-conjugated horseradish peroxidase (WGA-HRP), into the VPMcvm demonstrated that the thalamic pathway terminated mainly in a rostrocaudally narrow area in the dorsal part of granular insular cortex rostroventrally adjacent to the rostralmost part of the secondary somatosensory cortex (dGIrvs2). We also electrophysiologically confirmed that the dGIrvs2 received the proprioceptive inputs from JCMSs. To support the anatomical evidence of the VPMcvm-dGIrvs2 pathway, injections of a retrograde neuronal tracer Fluorogold into the dGIrvs2 demonstrated that the thalamic neurons projecting to the dGIrvs2 were confined in the VPMcvm and the parvicellular part of ventral posterior nucleus. In contrast, WGA-HRP injections into the lingual nerve area of core VPM demonstrated that axon terminals were mainly labeled in the core regions of the primary and secondary somatosensory cortices, which were far from the dGIrvs2. These results suggest that the dGIrvs2 is a specialized cortical region receiving the orofacial proprioceptive inputs. Functional contribution of the revealed JCMSs-VPMcvm-dGIrvs2 pathway to Tourette syndrome is also discussed.

Establishment and proteomic characterization of NCC-LMS1-C1, a novel cell line of primary leiomyosarcoma of the bone.

Leiomyosarcoma (LMS) is one of most aggressive mesenchymal malignancies that differentiate towards smooth muscle. The clinical outcome of LMS patients is poor; as such, there is an urgent need for novel therapeutic approaches. Experimental models such as patient-derived cell lines are invaluable tools for pre-clinical studies. In the present study, we established a stable cell line from the tumor tissue of a patient with a primary LMS of the bone. Despite the urgent need for novel therapeutic strategies in LMS, there are only a few LMS cell lines available in public cell banks, none of which are primary to the bone.

Leiomyosarcoma of maxilla: a case report with review of literature.

One challenging feature of head and neck pathology is that a dizzying array of spindle cell lesions occurs here which ranges all the way from reactive, very aggressive forms to malignant lesions. Leiomyosarcoma is one such malignant tumour of mesenchymal origin exhibiting smooth muscle differentiation; presenting generally nonspecific signs and symptoms. Here we present a case of leiomyosarcoma in a 21 year old female patient associated with single reddish pink swelling present in the posterior right maxillary tuberosity region with moderate facial asymmetry. On histopathological analysis, all the classic features were noted and diagnosis of a spindle cell neoplasm was made without any obscurity. There are many lesions like rhabdomyosarcoma, fibrosarcoma, liposarcoma, multiple peripheral nerve sheath tumour, angiosarcomas, melanomas, kaposi sarcoma, solitary fibrous tumors, etc., which mimic leiomyosarcoma clinically and histopathologically and therefore immunohistochemical staining with specific markers plays a vital role in arriving at a conclusive diagnosis.

BubR1 insufficiency impairs angiogenesis in aging and in experimental critical limb ischemic mice.

Budding uninhibited by benzimidazole-related 1 (BubR1), a cell cycle-related protein, is an essential component of the spindle checkpoint that regulates cell division. Mice in which BubR1 expression is reduced to 10% of the normal level display the phenotypic features of progeria. However, the role of BubR1 in vascular diseases and angiogenesis remains unknown. To investigate the influence of BubR1 on angiogenesis, we generated a low-null-BubR1-expressing (BubR1L/-) mouse strain with reduced BubR1 expression as low as 15% of the normal level without any abnormalities in appearance.

Plexiform Angiomyxoid Myofibroblastic Tumor of the Stomach: a Rare Case.

Plexiform angiomyxoid myofibroblastic tumor (PAMT) of the stomach is a very rare mesenchymal tumor of the gastrointestinal tract. We report a case of asymptomatic gastric PAMT that was pathologically confirmed after surgical resection. The tumor had a multinodular plexiform growth pattern, bland-looking spindle cells, and an Alcian blue-positive myxoid stromal matrix rich in small blood vessels. Immunohistochemistry analysis revealed that the tumor cells of the PAMT were positive for smooth muscle actin (SMA) and negative for c-kit, CD34, S-100 protein, epithelial membrane antigen (EMA), and desmin. PAMT should be differentiated from other submucosal tumors of the stomach by immunohistochemical findings. Considering the benign features of this tumor, observation without resection may be an option for the treatment of PAMT if the tumor is asymptomatic.

Development of Right-hemispheric Dominance of Inferior Parietal Lobule in Proprioceptive Illusion Task.

Functional lateralization can be an indicator of brain maturation. We have consistently shown that, in the adult brain, proprioceptive processing of muscle spindle afferents generating illusory movement of the right hand activates inferior frontoparietal cortical regions in a right-side dominant manner in addition to the cerebrocerebellar motor network. Here we provide novel evidence regarding the development of the right-dominant use of the inferior frontoparietal cortical regions in humans using this task. We studied brain activity using functional magnetic resonance imaging while 60 right-handed blindfolded healthy children (8-11 years), adolescents (12-15 years), and young adults (18-23 years) (20 per group) experienced the illusion. Adult-like right-dominant use of the inferior parietal lobule (IPL) was observed in adolescents, while children used the IPL bilaterally. In contrast, adult-like lateralized cerebrocerebellar motor activation patterns were already observable in children. The right-side dominance progresses during adolescence along with the suppression of the left-sided IPL activity that emerges during childhood. Therefore, the neuronal processing implemented in the adult's right IPL during the proprioceptive illusion task is likely mediated bilaterally during childhood, and then becomes right-lateralized during adolescence at a substantially later time than the lateralized use of the cerebrocerebellar motor system for kinesthetic processing.