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https://www.readbyqxmd.com/read/28717511/thalamotomy-for-essential-tremor-fda-approval-brings-brain-treatment-with-fus-to-the-clinic
#1
EDITORIAL
Paul S Fishman
No abstract text is available yet for this article.
2017: Journal of Therapeutic Ultrasound
https://www.readbyqxmd.com/read/28715648/tumor-suppressor-candidate-2-tusc2-fus-1-and-human-cancers
#2
Tadas Rimkus, Sherona Sirkisoon, Alexandra Harrison, Hui-Wen Lo
Tumor suppressor candidate 2 (TUSC2, also known as FUS1) was identified in 2000 as a candidate tumor suppressor gene located in a region on chromosome 3p21.3 that is homozygously deleted in some lung and breast cancers. The deletion is rare in lung and breast cancers, but is frequent in malignant pleural mesothelioma. Evidence to date indicates that TUSC2 behaves as a tumor suppressor in lung cancer; however, its role as a tumor suppressor for other tumor types has not been fully established. Loss of TUSC2 expression at the mRNA and protein levels has been reported in various cancers...
May 2017: Discovery Medicine
https://www.readbyqxmd.com/read/28710685/pericytes-extend-survival-of-als-sod1-mice-and-induce-the-expression-of-antioxidant-enzymes-in-the-murine-model-and-in-ipscs-derived-neuronal-cells-from-an-als-patient
#3
Giuliana Castello Coatti, Miriam Frangini, Marcos C Valadares, Juliana Plat Gomes, Natalia O Lima, Natale Cavaçana, Amanda F Assoni, Mayra V Pelatti, Alexander Birbrair, Antonio Carlos Pedroso de Lima, Julio M Singer, Francisco Marcelo M Rocha, Giovani Loiola Da Silva, Mario Sergio Mantovani, Lucia Inês Macedo-Souza, Merari F R Ferrari, Mayana Zatz
Amyotrophic Lateral Sclerosis (ALS) is one of the most common adult-onset motor neuron disease causing a progressive, rapid and irreversible degeneration of motor neurons in the cortex, brain stem and spinal cord. No effective treatment is available and cell therapy clinical trials are currently being tested in ALS affected patients. It is well known that in ALS patients, approximately 50% of pericytes from the spinal cord barrier are lost. In the central nervous system, pericytes act in the formation and maintenance of the blood-brain barrier, a natural defense that slows the progression of symptoms in neurodegenerative diseases...
July 14, 2017: Stem Cell Reviews
https://www.readbyqxmd.com/read/28708999/splicing-activation-by-rbfox-requires-self-aggregation-through-its-tyrosine-rich-domain
#4
Yi Ying, Xiao-Jun Wang, Celine K Vuong, Chia-Ho Lin, Andrey Damianov, Douglas L Black
Proteins of the Rbfox family act with a complex of proteins called the Large Assembly of Splicing Regulators (LASR). We find that Rbfox interacts with LASR via its C-terminal domain (CTD), and this domain is essential for its splicing activity. In addition to LASR recruitment, a low-complexity (LC) sequence within the CTD contains repeated tyrosines that mediate higher-order assembly of Rbfox/LASR and are required for splicing activation by Rbfox. This sequence spontaneously aggregates in solution to form fibrous structures and hydrogels, suggesting an assembly similar to the insoluble cellular inclusions formed by FUS and other proteins in neurologic disease...
July 13, 2017: Cell
https://www.readbyqxmd.com/read/28707655/-the-fus-protein-physiological-functions-and-a-role-in-amyotrophic-lateral-sclerosis
#5
A D Efimova, R K Ovchinnikov, A Yu Roman, A V Maltsev, V V Grigoriev, E A Kovrazhkina, V I Skvortsova
Certain forms of amyotrophic lateral sclerosis (ALS) are associated with an altered compartmentalization of FUS and its aggregation in the cytoplasm of motoneurons. FUS is a DNA/RNA-binding protein that is involved in DNA repair and the regulation of transcription, splicing, RNA transport, and local translation. Two theories have been proposed to explain the mechanism of the pathophysiological process in ALS. The theories attribute degeneration of motor neurons to either loss or gain of FUS function. The review describes the main physiological functions of FUS and considers evidence for each of the theories of ALS pathogenesis...
May 2017: Molekuliarnaia Biologiia
https://www.readbyqxmd.com/read/28701145/an-in-vitro-technique-to-identify-the-rna-binding-site-sequences-for-rna-binding-proteins
#6
SunKyung Choi, Chungoo Park, Kyoon Eon Kim, Kee K Kim
RNA-protein interactions play a major role in gene regulation. Although many techniques to analyze RNA-protein interactions have been developed, noteworthy challenges such as determining the RNA sequences that bind RNA-binding proteins (RBPs) remain unsolved. Here, we describe a novel technique using a 4-thio-uridine-incorporated RNA pool to identify the RBP-binding consensus sequences for RBPs produced by in vitro transcription and translation. To confirm the fidelity of this approach, we determined the consensus RBP-binding sequence for RBFOX2, UGC(A/U)(A/U)NU, which is very similar to the known RBFOX2-binding sequence, UGCAUG...
July 1, 2017: BioTechniques
https://www.readbyqxmd.com/read/28685560/short-term-efficacy-of-capacitive-resistive-diathermy-therapy-in-patients-with-low-back-pain-a-prospective-randomized-controlled-trial
#7
A Notarnicola, G Maccagnano, M F Gallone, I Covelli, S Tafuri, B Moretti
To compare the effectiveness of Doctor Tecar TherapyTM with that of laser therapy in the management of low back pain (LBP), a total of 60 patients with LBP were enrolled. The participants were randomly divided into two groups: a Tecar group (experimental group, 30 subjects), and a laser group (control group, 30 subjects). All the subjects received 10 sessions of therapy: one each day from Monday to Friday and the same again the following week. All the subjects were evaluated for pain (VAS) and disability (Roland and Morris score and Oswestry score) at baseline (T0), and 2 weeks (T1), 1 month (T2) and 2 months (T3) after the end of treatment...
April 2017: Journal of Biological Regulators and Homeostatic Agents
https://www.readbyqxmd.com/read/28681998/eribulin-regresses-a-doxorubicin-resistant-ewing-s-sarcoma-with-a-fus-erg-fusion-and-cdkn2a-deletion-for-the-patient-derived-orthotopic-xenograft-pdox-nude-mouse-model
#8
Kentaro Miyaki, Takashi Murakami, Tasuku Kiyuna, Kentaro Igarashi, Kei Kawaguchi, Yunfeng Li, Arun S Singh, Sarah M Dry, Mark A Eckardt, Yukihiko Hiroshima, Masashi Momiyama, Ryusei Matsuyama, Takashi Chishima, Itaru Endo, Fritz C Eilber, Robert M Hoffman
Ewing's sarcoma is a recalcitrant tumor greatly in need of more effective therapy. The aim of this study was to determine the efficacy of eribulin on a doxorubicin (DOX)-resistant Ewing's sarcoma patient derived orthotopic xenograft (PDOX) model. The Ewing's sarcoma PDOX model was previously established in the right chest wall of nude mice from the patient's right chest wall. In the previous study, the Ewing's sarcoma PDOX was resistant to DOX and sensitive to palbociclib and linsitinib. In the present study, the PDOX models were randomized into 3 groups when the tumor volume reached 60 mm(3) : G1, untreated control (n = 6); G2, DOX treated (n = 6), intraperitoneal (i...
July 6, 2017: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/28678751/association-between-cd40-rs1883832-and-immune-related-diseases-susceptibility-a-meta-analysis
#9
Jiaxuan Qin, Jinchun Xing, Rongfu Liu, Bin Chen, Yuedong Chen, Xuan Zhuang
BACKGROUND/OBJECTIVE: It has been reported that CD40 rs1883832 might be associated with immune-related diseases susceptibility. Owing to mixed and inconclusive results, we conducted a meta-analysis of case-control studies to summarize and clarify this association.Methods/main results: A systematic search of studies on the association between CD40 rs1883832 and immune-related diseases susceptibility was conducted in databases. Odds ratios and 95% confidence intervals were used to pool the effect size...
June 28, 2017: Oncotarget
https://www.readbyqxmd.com/read/28660843/rapidly-progressive-fronto-temporal-dementia-ftd-associated-with-frontotemporal-lobar-degeneration-ftld-in-the-presence-of-fused-in-sarcoma-fus-protein-a-rare-sporadic-and-aggressive-form-of-ftd
#10
Nicholas I Bradfield, Catriona McLean, John Drago, David G Darby, David Ames
Fronto-temporal dementia (FTD) associated with Fused in Sarcoma (FUS) protein accumulation is an uncommon cause of FTD with a distinct syndrome of young age onset behavioral variant FTD, without a family history of FTD and caudate atrophy. We present a sporadic case of a 61-year-old patient with mixed features of both behavioral variant FTD with later semantic language dissolution associated with pathologically proven FUS. He was older than usual for FUS pathology, his course was rapidly progressive, and he had atypical language features...
June 29, 2017: International Psychogeriatrics
https://www.readbyqxmd.com/read/28658382/hiv-aids-mortality-in-brazil-2000-2015-are-there-reasons-for-concern
#11
Mark Drew Crosland Guimarães, Mariângela Carneiro, Daisy Maria Xavier de Abreu, Elisabeth Barboza França
Introduction and objective: Mortality studies are essential for the monitoring of the HIV/AIDS epidemic. Quality and completeness of data from the mortality information system (SIM) require complementary approaches. Methods: Two sources of data were used to assess mortality trends due to HIV/AIDS in Brazil from 2000 and 2014/15: a) data from the SIM published by the Department of STDs, AIDS, and viral hepatitis, and b) Global Burden of Disease 2015 (GBD 2015) studies...
May 2017: Revista Brasileira de Epidemiologia, Brazilian Journal of Epidemiology
https://www.readbyqxmd.com/read/28657719/nanoscale-analysis-reveals-the-maturation-of-neurodegeneration-associated-protein-aggregates-grown-in-mrna-granules-then-released-by-stress-granule-proteins
#12
Sanae Abrakhi, Dmitry A Kretov, Bénédicte Desforges, Ioana Dobra, Ahmed Bouhss, David Pastré, Loic Hamon
TDP-43 and FUS are two mRNA-binding proteins associated with neurodegenerative diseases that form cytoplasmic inclusions with prion-like properties in affected neurons. Documenting the early stages of the formation of TDP-43 or FUS protein aggregates and the role of mRNA stress granules that are considered as critical intermediates for protein aggregation is therefore of interest to understand disease propagation. Here, we developed a single molecule approach via atomic force microscopy (AFM), which provides structural information out of reach by fluorescence microscopy...
July 25, 2017: ACS Nano
https://www.readbyqxmd.com/read/28648941/low-grade-fibromyxoid-sarcoma-clinical-morphologic-and-genetic-features
#13
REVIEW
Mustafa Mohamed, Cyril Fisher, Khin Thway
Low-grade fibromyxoid sarcoma (LGFMS) is a bland spindle cell neoplasm that typically arises in the deep soft tissues of the proximal extremities or trunk of young adults. The majority of LGFMS are characterized by a recurrent (7;16)(q34;p11) translocation, resulting in the FUS-CREB3L2 fusion gene, which generates a chimeric protein with transcriptional regulatory activity. Small numbers harbor a FUS-CREB3L1 fusion resulting from t(11;16)(p11;p11), whilst rare cases harbor the EWSR1-CREB3L1 fusion. LGFMS is of low to moderate cellularity and consists of bland spindle cells with small, angulated nuclei and scant, wispy cytoplasm, arranged in a whorled growth pattern and typically showing abrupt transition from myxoid to fibrous areas...
June 2017: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/28643383/multiecho-pseudo-golden-angle-stack-of-stars-thermometry-with-high-spatial-and-temporal-resolution-using-k-space-weighted-image-contrast
#14
Bryant T Svedin, Allison Payne, Bradley D Bolster, Dennis L Parker
PURPOSE: Implement and evaluate a 3D MRI method to measure temperature changes with high spatial and temporal resolution and large field of view. METHODS: A multiecho pseudo-golden angle stack-of-stars (SOS) sequence with k-space weighted image contrast (KWIC) reconstruction was implemented to simultaneously measure multiple quantities, including temperature, initial signal magnitude M(0), transverse relaxation time ( T2*), and water/fat images. Respiration artifacts were corrected using self-navigation...
June 22, 2017: Magnetic Resonance in Medicine: Official Journal of the Society of Magnetic Resonance in Medicine
https://www.readbyqxmd.com/read/28637688/fus-ddit3-fusion-protein-driven-igf-ir-signaling-is-a-therapeutic-target-in-myxoid-liposarcoma
#15
Marcel Trautmann, Jasmin Menzel, Christian Bertling, Magdalene Cyra, Ilka Isfort, Konrad Steinestel, Sandra Elges, Inga Grünewald, Bianca Altvater, Claudia Rossig, Stefan Fröhling, Susanne Hafner, Thomas Simmet, Pierre Åman, Eva Wardelmann, Sebastian Huss, Wolfgang Hartmann
Purpose: Myxoid liposarcoma is an aggressive disease with particular propensity to develop hematogenic metastases. Over 90% of myxoid liposarcoma are characterized by a reciprocal t(12;16)(q13;p11) translocation. The resulting chimeric FUS-DDIT3 fusion protein plays a crucial role in myxoid liposarcoma pathogenesis; however, its specific impact on oncogenic signaling pathways remains to be substantiated. We here investigate the functional role of FUS‑DDIT3 in IGF-IR/PI3K/Akt signaling driving myxoid liposarcoma pathogenesis...
June 21, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28631853/high-sensitivity-mr-acoustic-radiation-force-imaging-using-transition-band-balanced-steady-state-free-precession
#16
Yuan Zheng, Michael Marx, G Wilson Miller, Kim Butts Pauly
PURPOSE: MR acoustic radiation force imaging (MR-ARFI) provides a method to visualize the focal spot of a focused ultrasound (FUS) beam without introducing a significant temperature rise. With conventional spoiled MR-ARFI pulse sequences, the ARFI phase always equals the motion-encoded phase. In this work, MR-ARFI using transition band balanced steady-state free precession (bSSFP) is presented, which improves the sensitivity of MR-ARFI with high acquisition speed. THEORY AND METHODS: Motion-encoding gradients (MEG) are inserted into bSSFP sequences for MR-ARFI...
June 20, 2017: Magnetic Resonance in Medicine: Official Journal of the Society of Magnetic Resonance in Medicine
https://www.readbyqxmd.com/read/28622300/the-als-linked-e102q-mutation-in-sigma-receptor-1-leads-to-er-stress-mediated-defects-in-protein-homeostasis-and-dysregulation-of-rna-binding-proteins
#17
Alice Dreser, Jan Tilmann Vollrath, Antonio Sechi, Sonja Johann, Andreas Roos, Alfred Yamoah, Istvan Katona, Saeed Bohlega, Dominik Wiemuth, Yuemin Tian, Axel Schmidt, Jörg Vervoorts, Marc Dohmen, Cordian Beyer, Jasper Anink, Eleonora Aronica, Dirk Troost, Joachim Weis, Anand Goswami
Amyotrophic lateral sclerosis (ALS) is characterized by the selective degeneration of motor neurons (MNs) and their target muscles. Misfolded proteins which often form intracellular aggregates are a pathological hallmark of ALS. Disruption of the functional interplay between protein degradation (ubiquitin proteasome system and autophagy) and RNA-binding protein homeostasis has recently been suggested as an integrated model that merges several ALS-associated proteins into a common pathophysiological pathway...
June 16, 2017: Cell Death and Differentiation
https://www.readbyqxmd.com/read/28620838/modelling-fus-mislocalisation-in-an-in-vitro-model-of-innervated-human-muscle
#18
Sonja Prpar Mihevc, Mojca Pavlin, Simona Darovic, Marko Živin, Matej Podbregar, Boris Rogelj, Tomaz Mars
Degeneration of distal axons and neuromuscular junctions is an early feature in the pathology of amyotrophic lateral sclerosis (ALS), which culminates in motor neuron loss due to axon retraction and muscle atrophy. The complex interactions in the pathogenesis of ALS between motor neurons, muscle cells and accompanying glia require an appropriate experimental model. Here, we have defined a co-culture model based on human myotubes innervated by neurons from embryonic rat spinal cord explants to investigate the pathology and treatment of ALS...
June 15, 2017: Journal of Molecular Neuroscience: MN
https://www.readbyqxmd.com/read/28620717/multiple-variants-in-families-with-amyotrophic-lateral-sclerosis-and-frontotemporal-dementia-related-to-c9orf72-repeat-expansion-further-observations-on-their-oligogenic-nature
#19
Maria Pia Giannoccaro, Anna Bartoletti-Stella, Silvia Piras, Annalisa Pession, Patrizia De Massis, Federico Oppi, Michelangelo Stanzani-Maserati, Elena Pasini, Simone Baiardi, Patrizia Avoni, Piero Parchi, Rocco Liguori, Sabina Capellari
The C9orf72 repeat expansion (RE) is one of the most frequent causative mutations of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). However, it is still unclear how the C9orf72 RE can lead to a heterogeneous phenotype. Several reports have shown the coexistence of mutations in multiple ALS/FTD causative genes in the same family, suggesting an oligogenic etiology for ALS and FTD. Our aim was to investigate this phenomenon in an Italian group of ALS/FTD pedigrees carrying the C9orf72 RE...
July 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28617382/-dimebon-delays-the-onset-of-symptoms-of-fus-proteinopathy-in-transgenic-mice
#20
A V Maltsev, A V Deykin, R K Ovchinnikov, M M Chicheva, E A Kovrazhkina, O D Razinskaya, E V Bronovitsky, A I Budevich, Yu K Kirikovich, S O Bachurin, A A Ustyugov, V I Skvortsova
AIM: To evaluate an effect of dimebon on the onset of symptomatic stage in FUS.1-513 transgenic mice - a new genetic model of neurodegeneration, and to study the dynamics of disease progression in the terminal stage. MATERIAL AND METHODS: The study was carried out on males of line FUS1-513 with the contribution of genes from CD1 strains. Mice of the experimental group (n=28) received dimebon with water in the concentration of 70 mcg/ml starting from the 35th day of life...
2017: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
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