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https://www.readbyqxmd.com/read/27891578/dual-specificity-phosphatase-15-regulates-erk-activation-in-schwann-cells
#1
José F Rodríguez-Molina, Camila Lopez-Anido, Ki H Ma, Chongyu Zhang, Tyler Olson, Katharina N Muth, Matthias Weider, John Svaren
Schwann cells and oligodendrocytes are the myelinating cells of the peripheral and central nervous system, respectively. Despite having different myelin components and different transcription factors driving their terminal differentiation there are shared molecular mechanisms between the two. Sox10 is one common transcription factor required for several steps in development of myelinating glia. However, other factors are divergent as Schwann cells need the transcription factor Egr2/Krox20 and oligodendrocytes require Myrf...
November 27, 2016: Journal of Neurochemistry
https://www.readbyqxmd.com/read/27890619/primary-iris-leiomyoma
#2
Gabrielle A Yeaney, Sean Platt, Arun D Singh
Intraocular leiomyomas are uncommon and usually occur in the ciliary body. Primary leiomyoma of the iris is both rare and a difficult diagnosis to make, given melanocytic tumors are more common and may be amelanotic. The somewhat controversial diagnosis of iris leiomyoma requires further confirmation by immunohistochemistry and electron microscopy. Herein, we describe 58-year-old man with a 2 mm round translucent pink lesion of the iris. The tumor was excised by sector iridectomy. Immunohistochemistry showed positivity for both smooth muscle actin and desmin and negativity for S-100, HMB45, SOX10, Melan A, CD31, CD34 and h-caldesmon...
November 24, 2016: Survey of Ophthalmology
https://www.readbyqxmd.com/read/27864337/connexin-mediated-signalling-in-non-sensory-cells-is-crucial-for-the-development-of-sensory-inner-hair-cells-in-the-mouse-cochlea
#3
Stuart L Johnson, Federico Ceriani, Oliver Houston, Roman Polishchuk, Elena Polishchuk, Giulia Crispino, Veronica Zorzi, Fabio Mammano, Walter Marcotti
: Mutations in the genes encoding for gap junction proteins connexin 26 (Cx26) and connexin 30 (Cx30) have been linked to syndromic and non-syndromic hearing loss in mice and humans. The release of ATP from connexin hemichannels in cochlear non-sensory cells has been proposed to be the main trigger for action potential (AP) activity in immature sensory inner hair cells (IHCs), which is crucial for the refinement of the developing auditory circuitry. Using connexin knockout mice, we show that IHCs fire spontaneous APs even in the absence of ATP-dependent intercellular Ca(2+) signalling in the non-sensory cells...
November 18, 2016: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/27863645/identification-of-a-de-novo-mutation-of-sox10-in-a-chinese-patient-with-waardenburg-syndrome-type-iv
#4
Fenghe Liang, Min Zhao, Lynn Fan, Hongyan Zhang, Yang Shi, Rui Han, Chunyan Qu
OBJECTIVES: Waardenburg syndrome is a rare genetic disorder, characterized by the association of sensorineural hearing loss and pigmentation abnormalities. Four subtypes have been classified. The present study aimed to analyze the clinical feature and investigate the genetic cause for a Chinese case of Waardenburg type IV (WS4). METHODS: The patient and his family members were subjected to mutation detection in the candidate gene SOX10 by Sanger sequencing. RESULTS: The patient has the clinical features of WS4, including sensorineural hearing loss, bright blue irides, premature graying of the hair and Hirschsprung disease...
December 2016: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/27863528/oligodendrocyte-development-in-the-embryonic-tuberal-hypothalamus-and-the-influence-of-ascl1
#5
Candace M Marsters, Jessica M Rosin, Hayley F Thornton, Shaghayegh Aslanpour, Natasha Klenin, Grey Wilkinson, Carol Schuurmans, Quentin J Pittman, Deborah M Kurrasch
BACKGROUND: Although the vast majority of cells in our brains are glia, we are only beginning to understand programs governing their development, especially within the embryonic hypothalamus. In mice, gliogenesis is a protracted process that begins during embryonic stages and continues into the early postnatal period, with glial progenitors first producing oligodendrocyte precursor cells, which then differentiate into pro-oligodendrocytes, pro-myelinating oligodendrocytes, and finally, mature myelinating oligodendrocytes...
November 18, 2016: Neural Development
https://www.readbyqxmd.com/read/27857060/identification-of-transcription-factors-tfs-and-targets-involved-in-the-cholangiocarcinoma-cca-by-integrated-analysis
#6
L Yang, S Feng, Y Yang
The present study was designed to investigate the upstream transcription factors (TFs) and the signature genes in cholangiocarcinoma (CCA), providing better clues on the regulatory mechanisms and therapeutic applications. Gene expression data sets of CCA were searched in the Gene Expression Omnibus database for integrated analysis. Functional annotation of differently expressed genes (DEGs) was then conducted and the TFs were identified. Moreover, a global transcriptional regulatory network of TFs-targets was constructed...
November 18, 2016: Cancer Gene Therapy
https://www.readbyqxmd.com/read/27829444/potential-molecular-characteristics-in-situ-in-response-to-repetitive-uvb-irradiation
#7
Wenqi Chen, Jinhai Zhang
BACKGROUND: To identify molecular characteristics in situ in response to repetitive UVB (ultraviolet-B) irradiation. METHODS: Microarray data from the Gene Expression Omnibus were re-analyzed to identify DEGs (differentially expressed genes) between UVB-irradiated and non-irradiated skin biopsies. Enrichment and annotation analyses were performed respectively using DAVID, and TSGene and TAG databases. PPIs (protein-protein interactions) were analyzed using STRING, and miRNAs (microRNAs) and TFs (transcription factors) were predicted separately by miRNA-related databases and ENCODE...
November 10, 2016: Diagnostic Pathology
https://www.readbyqxmd.com/read/27821229/-clinicopathologic-features-of-dermal-nerve-sheath-myxoma-and-neurothekeoma-a-comparative-study
#8
H Ding, L L Wang, X L Xu, Q Y Lao I, L Yu, J Wang
Objective: To investigate the clinicopathologic characteristics and differential diagnosis of dermal nerve sheath myxoma (DNSM) and neurothekeoma (NTK). Methods: Clinical, pathological features and immunohistochemical profiles in 9 cases of DNSM and 8 cases of NTK were comparatively studied. The literature was reviewed. Results: The involved site of 9 DNSMs included the hand/fingers (n=3), ear (n=2), face, back, abdominal wall and waist (1 case each). Two of 8 NTKs arose in the forearm (n=2), one each in the nose, lip, shoulder, supraclavicular region, leg and hand...
November 8, 2016: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/27821050/stringent-comparative-sequence-analysis-reveals-sox10-as-a-putative-inhibitor-of-glial-cell-differentiation
#9
Chetna Gopinath, William D Law, José F Rodríguez-Molina, Arjun B Prasad, Lingyun Song, Gregory E Crawford, James C Mullikin, John Svaren, Anthony Antonellis
BACKGROUND: The transcription factor SOX10 is essential for all stages of Schwann cell development including myelination. SOX10 cooperates with other transcription factors to activate the expression of key myelin genes in Schwann cells and is therefore a context-dependent, pro-myelination transcription factor. As such, the identification of genes regulated by SOX10 will provide insight into Schwann cell biology and related diseases. While genome-wide studies have successfully revealed SOX10 target genes, these efforts mainly focused on myelinating stages of Schwann cell development...
November 7, 2016: BMC Genomics
https://www.readbyqxmd.com/read/27813711/a-scoring-system-to-predict-the-severity-of-hirschsprung-disease-at-diagnosis-and-its-correlation-with-molecular-genetics
#10
Raquel Núñez-Ramos, Raquel M Fernández, Miguel González-Velasco, Jesús Ruiz-Contreras, Enrique Galán-Gómez, Ramón Núñez-Núñez, Salud Borrego
OBJECTIVES: Hirschsprung disease (HSCR) has a wide range of severity. There are non-severe forms treated conservatively until surgery and severe forms that require an early stoma and prolonged hospitalization. Our objective was to establish a clinical scoring system to predict the severity of HSCR and to evaluate the possible existence of a clinical-genetic correlation. METHODS: We carried out a retrospective observational study including all HSCR cases treated in our hospital...
January 27, 2016: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/27809618/identification-of-upstream-transcription-factors-tfs-for-expression-signature-genes-in-breast-cancer
#11
Hongyan Zang, Ning Li, Yuling Pan, Jingguang Hao
Breast cancer is a common malignancy among women with a rising incidence. Our intention was to detect transcription factors (TFs) for deeper understanding of the underlying mechanisms of breast cancer. Integrated analysis of gene expression datasets of breast cancer was performed. Then, functional annotation of differentially expressed genes (DEGs) was conducted, including Gene Ontology (GO) enrichment and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway enrichment. Furthermore, TFs were identified and a global transcriptional regulatory network was constructed...
November 4, 2016: Gynecological Endocrinology
https://www.readbyqxmd.com/read/27805920/benign-glandular-schwannoma-with-ancient-change
#12
Alex C Holliday, Sean E Mazloom, Garrett C Coman, Michael S Kolodney, Rahul N Chavan, Douglas J Grider
Benign glandular schwannomas are rare and should be distinguished from malignant peripheral nerve sheath tumors with similar divergent tissue differentiation. The authors present a benign glandular schwannoma with ancient change that developed in the subcutis of a 46-year-old man's posterior calf. He lacked stigmata of neurofibromatosis type 1 (NF1). The glandular elements stained positively for epithelial membrane antigen and pancytokeratin. The spindled cells stained positively for SOX10 and S100 protein, supporting schwannian (neural crest) differentiation...
October 31, 2016: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/27796063/endogenous-gaba-controls-oligodendrocyte-lineage-cell-number-myelination-and-cns-internode-length
#13
Nicola B Hamilton, Laura E Clarke, I Lorena Arancibia-Carcamo, Eleni Kougioumtzidou, Moritz Matthey, Ragnhildur Káradóttir, Louise Whiteley, Linda H Bergersen, William D Richardson, David Attwell
Adjusting the thickness and internodal length of the myelin sheath is a mechanism for tuning the conduction velocity of axons to match computational needs. Interactions between oligodendrocyte precursor cells (OPCs) and developing axons regulate the formation of myelin around axons. We now show, using organotypic cerebral cortex slices from mice expressing eGFP in Sox10-positive oligodendrocytes, that endogenously released GABA, acting on GABAA receptors, greatly reduces the number of oligodendrocyte lineage cells...
October 31, 2016: Glia
https://www.readbyqxmd.com/read/27785726/accelerated-generation-of-oligodendrocyte-progenitor-cells-from-human-induced-pluripotent-stem-cells-by-forced-expression-of-sox10-and-olig2
#14
Pengyan Li, Mo Li, Xihe Tang, Shuyan Wang, Y Alex Zhang, Zhiguo Chen
Oligodendrocyte progenitor cells (OPCs) hold great promise for treatment of dysmyelinating disorders, such as multiple sclerosis and cerebral palsy. Recent studies on generation of human OPCs mainly use human embryonic stem cells (hESCs) or neural stem cells (NSCs) as starter cell sources for the differentiation process. However, NSCs are restricted in availability and the present method for generation of oligodendrocytes (OLs) from ESCs often requires a lengthy period of time. Here, we demonstrated a protocol to efficiently derive OPCs from human induced pluripotent stem cells (hiPSCs) by forced expression of two transcription factors (2TFs), Sox10 and Olig2...
October 25, 2016: Science China. Life Sciences
https://www.readbyqxmd.com/read/27784805/the-acute-inhibition-of-enteric-glial-metabolism-with-fluoroacetate-alters-calcium-signaling-hemichannel-function-and-the-expression-of-key-proteins
#15
Jonathon L McClain, Brian David Gulbransen
Glia play key roles in the regulation of neurotransmission in the nervous system. Fluoroacetate (FA) is a metabolic poison widely used to study glial functions by disrupting the tricarboxylic acid cycle enzyme aconitase. Despite the widespread use of FA, the effects of FA on essential glial functions such as calcium (Ca(2+)) signaling and hemichannel function remain unknown. Therefore, our goal was to specifically assess the impact of FA on essential glial cell functions that are involved with neurotransmission in the enteric nervous system...
October 26, 2016: Journal of Neurophysiology
https://www.readbyqxmd.com/read/27783941/mek-inhibitors-reverse-growth-of-embryonal-brain-tumors-derived-from-oligoneural-precursor-cells
#16
Katarzyna Modzelewska, Elena F Boer, Timothy L Mosbruger, Daniel Picard, Daniela Anderson, Rodney R Miles, Mitchell Kroll, William Oslund, Theodore J Pysher, Joshua D Schiffman, Randy Jensen, Cicely A Jette, Annie Huang, Rodney A Stewart
Malignant brain tumors are the leading cause of cancer-related deaths in children. Primitive neuroectodermal tumors of the CNS (CNS-PNETs) are particularly aggressive embryonal tumors of unknown cellular origin. Recent genomic studies have classified CNS-PNETs into molecularly distinct subgroups that promise to improve diagnosis and treatment; however, the lack of cell- or animal-based models for these subgroups prevents testing of rationally designed therapies. Here, we show that a subset of CNS-PNETs co-express oligoneural precursor cell (OPC) markers OLIG2 and SOX10 with coincident activation of the RAS/MAPK (mitogen-activated protein kinase) pathway...
October 25, 2016: Cell Reports
https://www.readbyqxmd.com/read/27768723/dissecting-the-origin-of-breast-cancer-subtype-stem-cell-and-the-potential-mechanism-of-malignant-transformation
#17
Xinyi Liu, Dongfei Feng, Dianming Liu, Shuyuan Wang, Xuexin Yu, Enyu Dai, Jing Wang, Lihong Wang, Wei Jiang
BACKGROUND: Breast cancer is the most common incident form of cancer in women including different subtypes. Cancer stem cells (CSCs) have been confirmed to exist in breast cancer. But the research on the origin of breast cancer subtype stem cells (BCSSCs) is still inadequate. METHODS: We identified the putative origin cells of BCSSCs through comparing gene signatures between BCSSCs and normal mammary cells from multiple perspectives: common signature, expression consistency, functional similarity and shortest path length...
2016: PloS One
https://www.readbyqxmd.com/read/27759704/sox10-positive-cells-within-scars-a-potential-diagnostic-pitfall
#18
Nathan T Harvey, Nathan J Acott, Benjamin A Wood
No abstract text is available yet for this article.
October 18, 2016: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/27759048/molecular-etiology-and-genotype-phenotype-correlation-of-chinese-han-deaf-patients-with-type-i-and-type-ii-waardenburg-syndrome
#19
Lianhua Sun, Xiaohua Li, Jun Shi, Xiuhong Pang, Yechen Hu, Xiaowen Wang, Hao Wu, Tao Yang
Waardenburg syndrome (WS) characterized by sensorineural hearing loss and pigmentary abnormalities is genetically heterogeneous and phenotypically variable. This study investigated the molecular etiology and genotype-phenotype correlation of WS in 36 Chinese Han deaf probands and 16 additional family members that were clinically diagnosed with WS type I (WS1, n = 8) and type II (WS2, n = 42). Mutation screening of six WS-associated genes detected PAX3 mutations in 6 (86%) of the 7 WS1 probands. Among the 29 WS2 probands, 13 (45%) and 10 (34%) were identified with SOX10 and MITF mutations, respectively...
October 19, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27718014/common-olfactory-ensheathing-glial-markers-in-the-developing-human-olfactory-system
#20
Karen Oprych, Daniel Cotfas, David Choi
The in situ immunocytochemical properties of olfactory ensheathing cells (OECs) have been well studied in several small to medium sized animal models including rats, mice, guinea pigs, cats and canines. However, we know very little about the antigenic characteristics of OECs in situ within the adult and developing human olfactory bulb and nerve roots. To address this gap in knowledge we undertook an immunocytochemical analysis of the 11-19 pcw human foetal olfactory system. Human foetal OECs in situ possessed important differences compared to rodents in the expression of key surface markers...
October 7, 2016: Brain Structure & Function
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