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https://www.readbyqxmd.com/read/28447247/the-role-of-peripheral-myelin-protein-2-in-remyelination
#1
Mark Stettner, Jennifer Zenker, Fabian Klingler, Fabian Szepanowski, Hans-P Hartung, Anne K Mausberg, Christoph Kleinschnitz, Roman Chrast, Bernd C Kieseier
The protein component of the myelin layer is essential for all aspects of peripheral nerves, and its deficiency can lead to structural and functional impairment. The presence of peripheral myelin protein 2 (P2, PMP2, FABP8, M-FABP) in Schwann cells has been known for decades and shown recently to be involved in the lipid homeostasis in the peripheral neural system. However, its precise role during de- and remyelination has yet to be elucidated. To this end, we assessed remyelination after sciatic nerve crush injury in vivo, and in an experimental de/remyelination ex vivo myelinating culture model in P2-deficient (P2 (-/-) ) and wild-type (WT) animals...
April 26, 2017: Cellular and Molecular Neurobiology
https://www.readbyqxmd.com/read/28446186/fingolimod-attenuates-experimental-autoimmune-neuritis-and-contributes-to-schwann-cell-mediated-axonal-protection
#2
Björn Ambrosius, Kalliopi Pitarokoili, Lisa Schrewe, Xiomara Pedreiturria, Jeremias Motte, Ralf Gold
BACKGROUND: Fingolimod, a sphingosine-1-phosphate receptor modulator with well-described immunomodulatory properties involving peripheral immune cell trafficking, was the first oral agent approved for the treatment of relapsing remitting multiple sclerosis. Analogous immunomodulatory treatment options for chronic peripheral autoimmune neuropathies are lacking. METHODS: We tested fingolimod in the animal model of experimental autoimmune neuritis in Lewis rat. Six to eight-week-old female rats were immunized with P2 peptide and from this day on treated with fingolimod...
April 26, 2017: Journal of Neuroinflammation
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
#3
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
https://www.readbyqxmd.com/read/28443238/a-case-report-of-giant-adrenal-ganglioneuroma
#4
Peijie Chen, Canbin Lin, Lu Jin, Yu Ding, Jian Peng, Zebo Chen, Shangqi Yang, Xiangming Mao, Yongqing Lai
Adrenal ganglioneuroma (AGN) is an extremely rare and benign entity comprising Schwann cells and ganglion cells. If the lesion approaches to 6 cm, laparoscopic resection is considered as relative contraindication. Now we present a giant adrenal AGN in a 25-year-old male patient. After abdominal computed tomography (CT) scanning, the patient underwent an exploratory laparotomy with right adrenalectomy and histopathological examination further confirmed the lesions as giant AGN, which measured 7 cm × 5 cm × 3...
July 2017: Urology Case Reports
https://www.readbyqxmd.com/read/28442932/granular-cell-tumor-in-breast-a-case-report
#5
Maria Castillo Lara, Antonia Martínez Herrera, Rafael Torrejón Cardoso, Daniel Maria Lubián López
Granular cell tumor (GCT) is a rare neoplasm of the soft tissues, and <1% of all GCTs are malignant. It usually appears in the tongue and sometimes may affect the female breast. Initially, GCT was considered to be a myogenic lesion affecting female breast (myoblastoma). Actually, it is assumed as a tumor originating from perineural or putative Schwann cells of peripheral nerves or their precursors that grows in the lobular breast tissue, due to the immunohistochemical features. Here, we review the importance of differentiating between this tumor and malignant breast carcinoma...
2017: Breast Cancer: Targets and Therapy
https://www.readbyqxmd.com/read/28440805/cell-transplantation-therapy-for-spinal-cord-injury
#6
REVIEW
Peggy Assinck, Greg J Duncan, Brett J Hilton, Jason R Plemel, Wolfram Tetzlaff
Spinal cord injury can lead to severe motor, sensory and autonomic dysfunction. Currently, there is no effective treatment for the injured spinal cord. The transplantation of Schwann cells, neural stem cells or progenitor cells, olfactory ensheathing cells, oligodendrocyte precursor cells and mesenchymal stem cells has been investigated as potential therapies for spinal cord injury. However, little is known about the mechanisms through which these individual cell types promote repair and functional improvements...
April 25, 2017: Nature Neuroscience
https://www.readbyqxmd.com/read/28440471/time-dependent-differential-expression-of-long-non-coding-rnas-following-peripheral-nerve-injury
#7
Bin Pan, Heng-Xing Zhou, Yi Liu, Jia-Yin Yan, Yao Wang, Xue Yao, Yan-Qiu Deng, Shu-Yi Chen, Lu Lu, Zhi-Jian Wei, Xiao-Hong Kong, Shi-Qing Feng
Long non-coding RNAs (lncRNAs) are widely accepted as key players in various biological processes. However, the roles of lncRNA in peripheral nerve regeneration remain completely unknown. Thus, in this study, we performed microarray analysis to measure lncRNA expression in the distal segment of the sciatic nerve at 0, 3, 7 and 14 days following injury. We identified 5,354 lncRNAs that were differentially expressed: 3,788 lncRNAs were differentially expressed between days 0 and 3; 3,314 lncRNAs were differentially expressed between days 0 and 7; and 2,400 lncRNAs were differentially expressed between days 0 and 14...
April 21, 2017: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/28432299/tangluoning-a-traditional-chinese-medicine-attenuates-in-vivo-and-in-vitro-diabetic-peripheral-neuropathy-through-modulation-of-perk-nrf2-pathway
#8
Xinwei Yang, Weijie Yao, Haolong Liu, Yanbin Gao, Renhui Liu, Liping Xu
Prolonged hyperglycemia-induced oxidative stress and endoplasmic reticulum stress have been demonstrated to play a key role in progression of diabetic peripheral neuropathy (DPN). PERK/ Nrf2 pathway plays a predominant role in oxidative and endoplasmic reticulum (ER) stress which is associated with cell survival. This study examined the modulation of the PERK/Nrf2 pathway and apoptosis by a traditional Chinese medicine Tangluoning (TLN) in streptozotocin-induced DPN rat models and the effects of serum TLN on the PERK/Nrf2 pathway, apoptosis, intracellular reactive oxygen species and mitochondrial membrane potential in Schwann cells cultured in 150 mM glucose...
April 21, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28427224/ponatinib-promotes-a-g1-cell-cycle-arrest-of-merlin-nf2-deficient-human-schwann-cells
#9
Alejandra M Petrilli, Jeanine Garcia, Marga Bott, Stephani Klingeman Plati, Christine T Dinh, Olena R Bracho, Denise Yan, Bing Zou, Rahul Mittal, Fred F Telischi, Xue-Zhong Liu, Long-Sheng Chang, D Bradley Welling, Alicja J Copik, Cristina Fernández-Valle
Neurofibromatosis type 2 (NF2) is a genetic syndrome that predisposes individuals to multiple benign tumors of the central and peripheral nervous systems, including vestibular schwannomas. Currently, there are no FDA approved drug therapies for NF2. Loss of function of merlin encoded by the NF2 tumor suppressor gene leads to activation of multiple mitogenic signaling cascades, including platelet-derived growth factor receptor (PDGFR) and SRC in Schwann cells. The goal of this study was to determine whether ponatinib, an FDA-approved ABL/SRC inhibitor, reduced proliferation and/or survival of merlin-deficient human Schwann cells (HSC)...
March 6, 2017: Oncotarget
https://www.readbyqxmd.com/read/28425952/antioxidant-and-anti-senescence-effect-of-metformin-on-mouse-olfactory-ensheathing-cells-moecs-may-be-associated-with-increased-brain-derived-neurotrophic-factor-levels-an-ex-vivo-study
#10
Agnieszka Śmieszek, Zuzanna Stręk, Katarzyna Kornicka, Jakub Grzesiak, Christine Weiss, Krzysztof Marycz
Metformin, the popular anti-diabetic drug was shown to exert multiple biological effects. The most recent metformin gained attention as an agent that mobilizes endogenous progenitor cells and enhances regenerative potential of organisms, for example by promoting neurogenesis. In the present study, we examined the role of metformin on mouse olfactory ensheathing cells (mOECs) derived from animals receiving metformin for eight weeks at a concentration equal to 2.8 mg/day. The mOECs expanded ex vivo were characterized in terms of their cellular phenotype, morphology, proliferative activity, viability and accumulation of oxidative stress factors...
April 20, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28412554/prompt-peripheral-nerve-regeneration-induced-by-a-hierarchically-aligned-fibrin-nanofiber-hydrogel
#11
Jinrong Du, Jianheng Liu, Shenglian Yao, Haiquan Mao, Jiang Peng, Xun Sun, Zheng Cao, Yongdong Yang, Bo Xiao, Yiguo Wang, Peifu Tang, Xiumei Wang
Fibrin plays a crucial role in peripheral nerve regeneration, which could occur spontaneously in the format of longitudinally oriented fibrin cables during the initial stage of nerve regeneration. This fibrin cable can direct migration and proliferation of Schwann cells and axonal regrowth, which is very important to nerve regeneration. In the present study, we prepared a three-dimensional hierarchically aligned fibrin nanofiber hydrogel (AFG) through electrospinning and molecular self-assembly to resemble the architecture and biological function of the native fibrin cable...
April 12, 2017: Acta Biomaterialia
https://www.readbyqxmd.com/read/28400720/superparamagnetic-iron-oxide-nanoparticle-mediated-forces-enhance-the-migration-of-schwann-cells-across-the-astrocyte-schwann-cell-boundary-in-vitro
#12
Liangliang Huang, Bing Xia, Zhongyang Liu, Quanliang Cao, Jinghui Huang, Zhuojing Luo
Schwann cells (SCs) are one of the most promising cellular candidates for the treatment of spinal cord injury. However, SCs show poor migratory ability within the astrocyte-rich central nervous system (CNS) environment and exhibit only limited integration with host astrocytes. Our strategy for improving the therapeutic potential of SCs was to magnetically drive SCs to migrate across the astrocyte-SC boundary to intermingle with astrocytes. SCs were firstly magnetized with poly-L-lysine-coated superparamagnetic iron oxide nanoparticles (SPIONs)...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/28392569/multichannel-polymer-scaffold-seeded-with-activated-schwann-cells-and-bone-mesenchymal-stem-cells-improves-axonal-regeneration-and-functional-recovery-after-rat-spinal-cord-injury
#13
Er-Zhu Yang, Guo-Wang Zhang, Jian-Guang Xu, Shuai Chen, Hua Wang, Liang-Liang Cao, Bo Liang, Xiao-Feng Lian
The adult mammalian CNS has a limited capacity to regenerate after traumatic injury. In this study, a combinatorial strategy to promote axonal regeneration and functional recovery after spinal cord injury (SCI) was evaluated in adult rats. The rats were subjected to a complete transection in the thoracic spinal cord, and multichannel scaffolds seeded with activated Schwann cells (ASCs) and/or rat bone marrow-derived mesenchymal stem cells (MSCs) were acutely grafted into the 3-mm-wide transection gap. At 4 weeks post-transplantation and thereafter, the rats receiving scaffolds seeded with ASCs and MSCs exhibited significant recovery of nerve function as shown by the Basso, Beattie and Bresnahan (BBB) score and electrophysiological test results...
April 10, 2017: Acta Pharmacologica Sinica
https://www.readbyqxmd.com/read/28387148/malignant-triton-tumor-malignant-peripheral-nerve-sheath-tumor-with-rhabdomyoblastic-differentiation-occurring-in-a-vascularized-free-flap-reconstruction-graft
#14
Roopa Ram, Jerad Gardner, Sindhura Alapati, Kedar Jambhekar, Tarun Pandey, Corey Montgomery, Richard Nicholas
Malignant peripheral nerve sheath tumor (MPNST) is a rare form of sarcoma arising from Schwann cells or pluripotent cells of the neural crest. Malignant triton tumor (MTT) is a subtype of MPNST with a component of malignant rhabdomyoblasts in addition to malignant Schwann cells. MPNST and MTT are both aggressive malignancies that most commonly arise from large deep neurofibromas in patients with neurofibromatosis type 1 (NF-1). However, sporadic non-NF-1 cases of MTT have also been reported in the literature...
April 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28384950/familial-schwannomatosis-a-diagnostic-challenge
#15
Sameer Ajit Mansukhani, Rajendra Prasad R Butala, Sunil H Shetty, Ravindra G Khedekar
Schwannomatosis is a disease characterized by the development of multiple benign tumours originating from Schwann cells. Schwannomatosis is a member of the family of diseases known as Neurofibromatosis (NF). Patients with Schwannomatosis develop multiple Schwannomas on cranial, spinal and peripheral nerves. We report a rare case of a 60-year-old female who presented with a painful swelling on the ulnar aspect of her distal forearm. She underwent an excisional biopsy for it; which was suggestive of a Schwannoma...
February 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28382305/pmp22-exon-4-deletion-causes-er-retention-of-pmp22-and-a-gain-of-function-allele-in-cmt1e
#16
David S Wang, Xingyao Wu, Yunhong Bai, Craig Zaidman, Tiffany Grider, John Kamholz, James R Lupski, Anne M Connolly, Michael E Shy
OBJECTIVE: To determine whether predicted fork stalling and template switching (FoSTeS) during mitosis deletes exon 4 in peripheral myelin protein 22 KD (PMP22) and causes gain-of-function mutation associated with peripheral neuropathy in a family with Charcot-Marie-Tooth disease type 1E. METHODS: Two siblings previously reported to have genomic rearrangements predicted to involve exon 4 of PMP22 were evaluated clinically and by electrophysiology. Skin biopsies from the proband were studied by RT-PCR to determine the effects of the exon 4 rearrangements on exon 4 mRNA expression in myelinating Schwann cells...
April 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/28381802/prophylactic-administration-of-aucubin-inhibits-paclitaxel-induced-mechanical-allodynia-via-the-inhibition-of-endoplasmic-reticulum-stress-in-peripheral-schwann-cells
#17
Tsugunobu Andoh, Daisuke Uta, Mitsuru Kato, Kazufumi Toume, Katsuko Komatsu, Yasushi Kuraishi
Paclitaxel is a chemotherapeutic agent that causes peripheral neuropathy as its major dose-limiting side effect. However, the peripheral neuropathy is difficult to manage. A study we recently conducted showed that repetitive administration of aucubin as a prophylactic inhibits paclitaxel-induced mechanical allodynia. However, the mechanisms underlying the anti-allodynic activity of aucubin, which is a major component of Plantaginis Semen, was unclear. In addition to mechanical allodynia, aucubin inhibited spontaneous and mechanical stimuli-induced firing in spinal dorsal horn neurons; however, catalpol, a metabolite of aucubin, did not show these effects...
2017: Biological & Pharmaceutical Bulletin
https://www.readbyqxmd.com/read/28377695/transplantation-of-pdgf-aa-overexpressing-oligodendrocyte-precursor-cells-promotes-recovery-in-rat-following-spinal-cord-injury
#18
Zong-Feng Yao, Ying Wang, Yu-Hong Lin, Yan Wu, An-You Zhu, Rui Wang, Lin Shen, Jin Xi, Qi Qi, Zhi-Quan Jiang, He-Zuo Lü, Jian-Guo Hu
Our previous study showed that Schwann cells (SCs) promote survival, proliferation and migration of co-transplanted oligodendrocyte progenitor cells (OPCs) and neurological recovery in rats with spinal cord injury (SCI). A subsequent in vitro study confirmed that SCs modulated OPC proliferation and migration by secreting platelet-derived growth factor (PDGF)-AA and fibroblast growth factor-2 (FGF)-2. We also found that PDGF-AA stimulated OPC proliferation and their differentiation into oligodendrocytes (OLs) at later stages...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/28372342/a-rare-case-of-aggressive-melanotic-schwannoma-occurred-in-spinal-nerve-of-a-59-year-old-male
#19
Sung-Eun Choi, Yoon Jin Cha, Jisup Kim, Hyunseo Cha, Jayeong Seo, Sung-Uk Kuh, Sung-Jun Kim, Se Hoon Kim
Melanotic schwannoma (MS) is a rare variant of nerve sheath neoplasm that shows ultrastructural and immunophenotypical features of Schwann cells but also has cytoplasmic melanosomes and is reactive for melanocytic markers as well. Unlike conventional schwannoma, which is totally benign, MS has an unpredictable prognosis and is thought to have low-malignant potential. Herein, we present a rare case of recurrent MS in lumbar spine of a 59-year-old male.
April 4, 2017: Journal of Pathology and Translational Medicine
https://www.readbyqxmd.com/read/28369367/collagen-xiii-secures-pre-and-postsynaptic-integrity-of-the-neuromuscular-synapse
#20
Heli Härönen, Zarin Zainul, Hongmin Tu, Nikolay Naumenko, Raija Sormunen, Ilkka Miinalainen, Anastasia Shakirzyanova, Tuomo Oikarainen, Azat Abdullin, Paula Martin, Sabrina Santoleri, Jari Koistinaho, Israel Silmanl, Rashid Giniatullin, Michael A Fox, Anne Heikkinen, Taina Pihlajaniemi
Both transmembrane and extracellular cues, one of which is collagen XIII, regulate formation and function of the neuromuscular synapse, and their absence results in myasthenia. We show that the phenotypical changes in collagen XIII knock-out mice are milder than symptoms in human patients, but the Col13a1-/- mice recapitulate major muscle findings of congenital myasthenic syndrome type 19 and serve as a disease model. In the lack of collagen XIII neuromuscular synapses do not reach full size, alignment, complexity and function resulting in reduced muscle strength...
March 24, 2017: Human Molecular Genetics
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