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https://www.readbyqxmd.com/read/28620864/adipose-tissue-derived-stromal-cells-adsc-express-oligodendrocyte-and-myelin-markers-but-they-do-not-function-as-oligodendrocytes
#1
Lara Vellosillo, Maria Paz Muñoz, Carlos Luis Paíno
Mesenchymal cells cultured from the vasculo-stromal fraction of adipose tissue (ADSC) show adult stem cell characteristics and several groups have claimed generating neural cells from them. However, we have observed that many markers commonly used for the identification of neural cells are spontaneously expressed by ADSC in culture. In the present study, we have examined the expression of characteristic oligodendrocyte molecules in cultured ADSC, aiming to test if myelinating cells could be generated from accessible non-neural adult tissues...
June 15, 2017: Histochemistry and Cell Biology
https://www.readbyqxmd.com/read/28607484/rare-cell-variability-and-drug-induced-reprogramming-as-a-mode-of-cancer-drug-resistance
#2
Sydney M Shaffer, Margaret C Dunagin, Stefan R Torborg, Eduardo A Torre, Benjamin Emert, Clemens Krepler, Marilda Beqiri, Katrin Sproesser, Patricia A Brafford, Min Xiao, Elliott Eggan, Ioannis N Anastopoulos, Cesar A Vargas-Garcia, Abhyudai Singh, Katherine L Nathanson, Meenhard Herlyn, Arjun Raj
Therapies that target signalling molecules that are mutated in cancers can often have substantial short-term effects, but the emergence of resistant cancer cells is a major barrier to full cures. Resistance can result from secondary mutations, but in other cases there is no clear genetic cause, raising the possibility of non-genetic rare cell variability. Here we show that human melanoma cells can display profound transcriptional variability at the single-cell level that predicts which cells will ultimately resist drug treatment...
June 15, 2017: Nature
https://www.readbyqxmd.com/read/28605832/comparison-of-reprogramming-methods-for-generation-of-induced-oligodendrocyte-precursor-cells
#3
Eun-Hye Lee, Chang-Hwan Park
Direct conversion by trans-differentiation is of growing interest in cell therapy for incurable diseases. The efficiency of cell reprogramming and functionality of converted cells are important considerations in cell transplantation therapy. Here, we compared two representative protocols for the generation of induced-oligodendrocyte progenitor cells (iOPCs) from mouse and rat fibroblasts. Then, we showed that induction of Nkx6.2, Olig2, and Sox10 (NOS) was more effective in mouse fibroblasts and that induction of Olig2, Sox10, and Zfp536 (OSZ) was more effective at reprogramming iOPCs from rat fibroblasts...
June 14, 2017: Biomolecules & Therapeutics
https://www.readbyqxmd.com/read/28605142/aberrant-expression-of-fli-1-in-melanoma
#4
Nisha Ramani, Phyu P Aung, Wen-Jen Hwu, Priyadharsini Nagarajan, Michael T Tetzlaff, Jonathan L Curry, Doina Ivan, Victor G Prieto, Carlos A Torres-Cabala
FLI-1 (Friend leukemia integration site 1) nuclear transcription factor has been proposed as a suitable tool in the differential diagnosis of small round cell sarcomas. It has also been described as nuclear marker of endothelial differentiation. Expression of FLI-1 has been demonstrated in Ewing's sarcoma/ primitive neuroectodermal tumor (ES/PNET) and vascular neoplasms. In the present study, we describe two cases of metastatic melanoma with small round blue cell morphology that showed strong nuclear expression of FLI-1...
June 12, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28601899/sensory-innervation-of-the-anal-canal-and-anorectal-line-in-hirschsprung-s-disease-histological-evidence-from-mouse-models
#5
Masahiro Takeda, Katsumi Miyahara, Chihiro Akazawa, Geoffrey J Lane, Atsuyuki Yamataka
AIM: We used non-Hirschsprung's disease (HD) Sox10-Venus Transgenic mice (non-HDSV-mice), an endothelin receptor-B knockout mouse model of HD (HD-mice), and C57B6C3 wild controls (C-mice) to identify the correlation between the anorectal line (ARL) and successful transanal pull-through (TAPT). METHODS: In non-HDSV-mice, intestinal neural crest-derived cells can be visualized with Venus,-a green fluorescent protein-without histochemical staining. We exposed the anal canal in each non-HDSV-mouse and marked the ARL directly with red ink...
June 10, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28601552/deletion-of-p2-promoter-of-gjb1-gene-a-cause-of-charcot-marie-tooth-disease
#6
R Kulshrestha, S Burton-Jones, T Antoniadi, M Rogers, Z Jaunmuktane, S Brandner, N Kiely, R Manuel, T Willis
X-linked Charcot-Marie-Tooth disease (CMT) is the second most common cause of CMT, and is usually caused by mutations in the gap junction protein beta 1 (GJB1) gene. This gene has nerve specific P2 promoter that work synergistically with SOX10 and EGR2 genes to initiate transcription. Mutation in this region is known to cause Schwann cell dysfunction. A single large family of X linked peripheral neuropathy was identified in our practice. Next generation sequencing for targeted panel assay identified an upstream exon-splicing deletion identified extending from nucleotide c...
May 4, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28570006/primary-conjunctival-myxoma-case-series-and-review-of-the-literature
#7
Dolores Ríos Y Valles-Valles, Ivette Hernández-Ayuso, Héctor A Rodríguez-Martínez, Armando Medina-Cruz, Guillermo Salcedo-Casillas, Abelardo A Rodríguez-Reyes
AIMS: Myxomas are benign soft tissue tumors resembling primitive mesenchyme. They rarely involve ocular structures and have been recognized in conjunctiva, eyelids, cornea and orbit. The purpose of this study is to describe the clinicopathological features of 7 patients with primary conjunctival myxoma seen at Dr. Luis Sánchez Bulnes Hospital an ophthalmologic referral center in Mexico City. MATERIALS AND METHODS: We reviewed the clinical, histochemical and immunohistochemical studies of patients with documented myxoma of the conjunctiva diagnosed in our hospital...
June 1, 2017: Histopathology
https://www.readbyqxmd.com/read/28556023/index-report-of-cutaneous-angiosarcoma-with-strong-positivity-for-tyrosinase-mimicking-melanoma-with-further-evaluation-of-melanocytic-markers-in-a-large-angiosarcoma-series
#8
Alicia Leon-Castillo, John S A Chrisinger, Gauri Panse, Rashmi T Samdani, Davis R Ingram, Vinod Ravi, Victor G Prieto, Wei-Lien Wang, Alexander J Lazar
Cutaneous angiosarcoma can be challenging to diagnose particularly when poorly vasoformative and on biopsies. We report a case of a cutaneous angiosarcoma with strong positivity for tyrosinase, the first to our knowledge, initially misdiagnosed as melanoma. We subsequently evaluated the reactivity of panmelanocytic cocktail (tyrosinase, HMB-45 and melan-A), SOX10, tyrosinase and MITF in a large tissue microarray (TMA) of angiosarcoma. The TMA included 142 cases of angiosarcomas (29 cutaneous, 22 primary breast, 41 post-radiation breast, 15 visceral, 26 deep soft tissue and bone, 5 chronic lymphedema-associated, and 4 angiosarcomas arising in other sarcomas)...
May 27, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28551330/histopathologic-evaluation-of-atypical-neurofibromatous-tumors-and-their-transformation-into-malignant-peripheral-nerve-sheath-tumor-in-neurofibromatosis-1-patients-a-consensus-overview
#9
Markku M Miettinen, Cristina R Antonescu, Christopher D M Fletcher, Aerang Kim, Alexander J Lazar, Martha M Quezado, Karlyne M Reilly, Anat Stemmer-Rachamimov, Douglas R Stewart, David Viskochil, Brigitte Widemann, Arie Perry
Neurofibromatosis 1 (NF1) patients develop multiple neurofibromas, with 8-15% of patients experiencing malignant peripheral nerve sheath tumor (MPNST) during their lifetime. Prediction of transformation, typically from plexiform neurofibroma, is clinically and histologically challenging. In this overview, following a consensus meeting in October 2016, we outline the histopathologic features and molecular mechanisms involved in the malignant trans-formation of neurofibromas. Nuclear atypia alone is generally insignificant...
May 24, 2017: Human Pathology
https://www.readbyqxmd.com/read/28549040/nordiqc-assessments-of-sox10-immunoassays
#10
Rasmus Røge, Søren Nielsen, Michael Bzorek, Mogens Vyberg
No abstract text is available yet for this article.
May 25, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28548128/genomic-profiling-of-breast-secretory-carcinomas-reveals-distinct-genetics-from-other-breast-cancers-and-similarity-to-mammary-analog-secretory-carcinomas
#11
Gregor Krings, Nancy M Joseph, Gregory R Bean, David Solomon, Courtney Onodera, Eric Talevich, Iwei Yeh, James P Grenert, Elizabeth Hosfield, Emily D Crawford, Richard C Jordan, Annemieke van Zante, Charles Zaloudek, Sandra J Shin, Yunn-Yi Chen
Secretory carcinomas of the breast are rare tumors with distinct histologic features, recurrent t(12;15)(p13;q25) translocation resulting in ETV6-NTRK3 gene fusion and indolent clinical behavior. Mammary analog secretory carcinomas arising in other sites are histopathologically similar to the breast tumors and also harbor ETV6-NTRK3 fusions. Breast secretory carcinomas are often triple (estrogen and progesterone receptor, HER2) negative with a basal-like immunophenotype. However, genomic studies are lacking, and whether these tumors share genetic features with other basal and/or triple negative breast cancers is unknown...
May 26, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28544110/waardenburg-syndrome-a-rare-cause-of-inherited-neuropathy-due-to-sox10-mutation
#12
Petya Bogdanova-Mihaylova, Michael D Alexander, Raymond Pj Murphy, Sinéad M Murphy
Waardenburg syndrome (WS) is a rare disorder comprising sensorineural deafness and pigmentation abnormalities. Four distinct subtypes are defined based on the presence or absence of additional symptoms. Mutations in six genes have been described in WS. SOX10 mutations are usually associated with a more severe phenotype of WS with peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy and Hirschsprung disease. Here we report a 32 year-old man with a novel heterozygous missense variant in SOX10 gene, who presented with congenital deafness, Hirschsprung disease, iris heterochromia, foot deformity and intermediate conduction velocity length-dependent sensorimotor neuropathy...
May 22, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28534044/hsp-and-deafness-neurocristopathy-caused-by-a-novel-mosaic-sox10-mutation
#13
Sandra Donkervoort, Diana Bharucha-Goebel, Pomi Yun, Ying Hu, Payam Mohassel, Ahmet Hoke, Wadih M Zein, Daniel Ezzo, Andrea M Atherton, Ann C Modrcin, Majed Dasouki, A Reghan Foley, Carsten G Bönnemann
OBJECTIVE: To identify the underlying genetic cause in 2 sisters affected with progressive lower extremity spasticity, neuropathy, and early-onset deafness. METHODS: Whole-exome sequencing was performed, and segregation testing of variants was investigated using targeted Sanger sequencing. An inherited paternal mosaic mutation was further evaluated through quantitative analysis of the ratio of mutant vs wild-type allele in genomic DNA from various tissues, including blood, dermal fibroblasts, and saliva...
June 2017: Neurology. Genetics
https://www.readbyqxmd.com/read/28512649/purification-and-characterization-of-schwann-cells-from-adult-human-skin-and-nerve
#14
Jo Anne Stratton, Ranjan Kumar, Sarthak Sinha, Prajay Shah, Morgan Stykel, Yuval Shapira, Rajiv Midha, Jeff Biernaskie
Despite its modest capacity for regeneration, peripheral nervous system injury often results in significant long-term disability. Supplementing peripheral nervous system injury with autologous Schwann cells (SCs) may serve to rejuvenate the postinjury environment to enhance regeneration and ultimately improve functional outcomes. However, human nerve-derived SC (hN-SC) collection procedures require invasive surgical resection. Here, we describe the characterization of SCs from adult human skin (hSk-SCs) of four male donors ranging between 27 and 46 years old...
May 2017: ENeuro
https://www.readbyqxmd.com/read/28510278/tfg-met-fusion-in-an-infantile-spindle-cell-sarcoma-with-neural-features
#15
Uta Flucke, Max M van Noesel, Marc Wijnen, Lei Zhang, Chun-Liang Chen, Yun-Shao Sung, Cristina R Antonescu
An increasing number of congenital and infantile sarcomas displaying a primitive, monomorphic spindle cell phenotype have been characterized to harbor recurrent gene fusions, including infantile fibrosarcoma and congenital spindle cell rhabdomyosarcoma. Here we report an unusual spindle cell sarcoma presenting as a large and infiltrative pelvic soft tissue mass in a 4-month-old girl, which revealed a novel TFG-MET gene fusion by whole transcriptome RNA sequencing. The tumor resembled the morphology of an infantile fibrosarcoma with both fascicular and patternless growth, however, it expressed strong S100 protein immunoreactivity, while lacking SOX10 staining and retaining H3K27me3 expression...
May 16, 2017: Genes, Chromosomes & Cancer
https://www.readbyqxmd.com/read/28500913/myb-fusions-and-cd-markers-as-tools-for-authentication-and-purification-of-cancer-stem-cells-from-salivary-adenoid-cystic-carcinoma
#16
Alex Panaccione, Yi Zhang, Molly Ryan, Christopher A Moskaluk, Karen S Anderson, Wendell G Yarbrough, Sergey V Ivanov
Cancer stem cells (CSC) are considered the major cause of aggressive tumor behavior, recurrence, metastases, and resistance to radiation, making them an attractive therapeutic target. However, isolation of CSC from tumor tissue and their characterization are challenging due to uncertainty about their molecular markers and conditions for their propagation. Adenoid cystic carcinoma (ACC), which arises predominantly in the salivary glands, is a slow-growing but relentless tumor that frequently invades nerves and metastasizes...
May 2017: Stem Cell Research
https://www.readbyqxmd.com/read/28473753/mechanical-strain-promotes-oligodendrocyte-differentiation-by-global-changes-of-gene-expression
#17
Anna Jagielska, Alexis L Lowe, Ekta Makhija, Liliana Wroblewska, Jochen Guck, Robin J M Franklin, G V Shivashankar, Krystyn J Van Vliet
Differentiation of oligodendrocyte progenitor cells (OPC) to oligodendrocytes and subsequent axon myelination are critical steps in vertebrate central nervous system (CNS) development and regeneration. Growing evidence supports the significance of mechanical factors in oligodendrocyte biology. Here, we explore the effect of mechanical strains within physiological range on OPC proliferation and differentiation, and strain-associated changes in chromatin structure, epigenetics, and gene expression. Sustained tensile strain of 10-15% inhibited OPC proliferation and promoted differentiation into oligodendrocytes...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/28455697/the-differentiation-of-skin-mesenchymal-stem-cells-towards-a-schwann-cell-phenotype-impact-of-sigma-1-receptor-activation
#18
L Saulite, E Vavers, L Zvejniece, M Dambrova, U Riekstina
Neural crest stem cells (NCSCs) are the source of mature Schwann cells in the peripheral nervous system (PNS). The NCSC population resides in the bulge of hair follicles and in the dermis. Recently, it was shown that 2-3% of the human dermis mesenchymal stem cell (MSC) population expresses the NCSC marker CD271, thus enabling the use of skin MSCs for studying Schwann cell differentiation in vitro. The aims of this study were to establish a protocol for human skin MSC differentiation towards Schwann cell-like cells (SC-lcs) and to analyse the expression of sigma-1 receptor (S1R) in SC-lcs...
April 28, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28449850/migration-pathways-of-sacral-neural-crest-during-development-of-lower-urogenital-tract-innervation
#19
Carrie B Wiese, Karen K Deal, Sara J Ireland, V Ashley Cantrell, E Michelle Southard-Smith
The migration and fate of cranial and vagal neural crest-derived progenitor cells (NCPCs) have been extensively studied; however, much less is known about sacral NCPCs particularly in regard to their distribution in the urogenital system. To construct a spatiotemporal map of NCPC migration pathways into the developing lower urinary tract, we utilized the Sox10-H2BVenus transgene to visualize NCPCs expressing Sox10. Our aim was to define the relationship of Sox10-expressing NCPCs relative to bladder innervation, smooth muscle differentiation, and vascularization through fetal development into adulthood...
April 24, 2017: Developmental Biology
https://www.readbyqxmd.com/read/28443644/a-reciprocal-regulatory-loop-between-taz-yap-and-g-protein-g%C3%AE-s-regulates-schwann-cell-proliferation-and-myelination
#20
Yaqi Deng, Lai Man Natalie Wu, Shujun Bai, Chuntao Zhao, Haibo Wang, Jincheng Wang, Lingli Xu, Masahide Sakabe, Wenhao Zhou, Mei Xin, Q Richard Lu
Schwann cell (SC) myelination in the peripheral nervous system is essential for motor function, and uncontrolled SC proliferation occurs in cancer. Here, we show that a dual role for Hippo effectors TAZ and YAP in SC proliferation and myelination through modulating G-protein expression and interacting with SOX10, respectively. Developmentally regulated mutagenesis indicates that TAZ/YAP are critical for SC proliferation and differentiation in a stage-dependent manner. Genome-wide occupancy mapping and transcriptome profiling reveal that nuclear TAZ/YAP promote SC proliferation by activating cell cycle regulators, while targeting critical differentiation regulators in cooperation with SOX10 for myelination...
April 26, 2017: Nature Communications
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