scholarly journals A Mouse Model of Schwartz-Jampel Syndrome Reveals Myelinating Schwann Cell Dysfunction with Persistent Axonal Depolarization in Vitro and Distal Peripheral Nerve Hyperexcitability When Perlecan Is Lacking

2012 ◽  
Vol 180 (5) ◽  
pp. 2040-2055 ◽  
Author(s):  
Marie Bangratz ◽  
Nadège Sarrazin ◽  
Jérôme Devaux ◽  
Désirée Zambroni ◽  
Andoni Echaniz-Laguna ◽  
...  
Keyword(s):  
Schwann Cell ◽  
Mouse Model ◽  
Peripheral Nerve ◽  
Cell Dysfunction ◽  
Peripheral Nerve Hyperexcitability ◽  
Myelinating Schwann Cell

scholarly journals Interleukin-4 Regulates the Expression of CD209 and Subsequent Uptake of Mycobacterium leprae by Schwann Cells in Human Leprosy

2010 ◽  
Vol 78 (11) ◽  
pp. 4634-4643 ◽  
Author(s):  
Rosane M. B. Teles ◽  
Stephan R. Krutzik ◽  
Maria T. Ochoa ◽  
Rosane B. Oliveira ◽  
Euzenir N. Sarno ◽  
...  
Keyword(s):  
Schwann Cell ◽  
Peripheral Nerve ◽  
Schwann Cells ◽  
Primary Cultures ◽  
Mycobacterium Leprae ◽  
Microbial Pathogens ◽  
Interleukin 4 ◽  
Common Mechanism ◽  
Specific Cell

ABSTRACT The ability of microbial pathogens to target specific cell types is a key aspect of the pathogenesis of infectious disease. Mycobacterium leprae, by infecting Schwann cells, contributes to nerve injury in patients with leprosy. Here, we investigated mechanisms of host-pathogen interaction in the peripheral nerve lesions of leprosy. We found that the expression of the C-type lectin, CD209, known to be expressed on tissue macrophages and to mediate the uptake of M. leprae, was present on Schwann cells, colocalizing with the Schwann cell marker, CNPase (2′,3′-cyclic nucleotide 3′-phosphodiesterase), along with the M. leprae antigen PGL-1 in the peripheral nerve biopsy specimens. In vitro, human CD209-positive Schwann cells, both from primary cultures and a long-term line, have a higher binding of M. leprae compared to CD209-negative Schwann cells. Interleukin-4, known to be expressed in skin lesions from multibacillary patients, increased CD209 expression on human Schwann cells and subsequent Schwann cell binding to M. leprae, whereas Th1 cytokines did not induce CD209 expression on these cells. Therefore, the regulated expression of CD209 represents a common mechanism by which Schwann cells and macrophages bind and take up M. leprae, contributing to the pathogenesis of leprosy.

The Distribution of Electron-Dense Tracers in Peripheral Nerve Fibres

1971 ◽  
Vol 8 (2) ◽  
pp. 541-555
Author(s):  
SUSAN M. HALL ◽  
P. L. WILLIAMS
Keyword(s):  
Schwann Cell ◽  
Peripheral Nerve ◽  
Myelin Sheath ◽  
External Surface ◽  
Nerve Fibres ◽  
Cell Cytoplasm ◽  
Line Region ◽  
Periaxonal Space

Two electron-dense tracers, ferritin and lanthanum, have been administered to peripheral nerve fibres, and their uptake has been studied ultrastructurally. It was found that the perineurium was an effective barrier to ferritin in vivo, and the tracer was subsequently injected sub-perineurially. Ferritin uptake over a 120-min period was confined to occasional phagocytic vesicles in perineurial and Schwann cells, and to the nodal gap substance and paranodal periaxonal space. No uptake was observed in the myelin sheath, incisural intraperiod line gap, or in the axoplasm. Soaking fibres in ferritin in vitro resulted in a more generalized cytoplasmic and axoplasmic uptake, although the myelin sheath and Schmidt-Lanterman incisures remained devoid of the tracer. Lanthanum nitrate, included in the fixative solution, delineated the patent incisural intraperiod line gap, and outlined the external surface of the terminal loops of nodal Schwann cell cytoplasm, and the paranodal Schwann cell-axolemmal junction. Unlike ferritin, La3+ penetrated the myelin sheath, being usually confined to the intraperiod line region of the outer lamellae, where it was associated with a widening of the lamellar unit, and an apparent splitting of the intraperiod line. The results are discussed with regard to distribution of extracellular space in peripheral nerve fibres.

Optimization of Schwann Cell Adhesion in Response to Shear Stress in an in Vitro Model for Peripheral Nerve Tissue Engineering

2003 ◽  
Vol 9 (2) ◽  
pp. 233-241 ◽  
Author(s):  
Dara Chafik ◽  
David Bear ◽  
Phong Bui ◽  
Arush Patel ◽  
Neil F. Jones ◽  
...  
Keyword(s):  
Tissue Engineering ◽  
Shear Stress ◽  
Cell Adhesion ◽  
Schwann Cell ◽  
Peripheral Nerve ◽  
In Vitro Model ◽  
Nerve Tissue ◽  
Nerve Tissue Engineering ◽  
Vitro Model

scholarly journals Zonisamide Enhances Neurite Elongation of Primary Motor Neurons and Facilitates Peripheral Nerve Regeneration In Vitro and in a Mouse Model

PLoS ONE ◽  
2015 ◽  
Vol 10 (11) ◽  
pp. e0142786 ◽  
Author(s):  
Hideki Yagi ◽  
Bisei Ohkawara ◽  
Hiroaki Nakashima ◽  
Kenyu Ito ◽  
Mikito Tsushima ◽  
...  
Keyword(s):  
Mouse Model ◽  
Peripheral Nerve ◽  
Nerve Regeneration ◽  
Motor Neurons ◽  
Peripheral Nerve Regeneration ◽  
Neurite Elongation ◽  
Regeneration In Vitro

scholarly journals Correction: Promoting Myelination in an In Vitro Mouse Model of the Peripheral Nerve System: The Effect of Wine Ingredients

PLoS ONE ◽  
2013 ◽  
Vol 8 (9) ◽  
Author(s):  
Mark Stettner ◽  
Kathleen Wolffram ◽  
Anne K. Mausberg ◽  
Philipp Albrecht ◽  
Angelika Derksen ◽  
...  
Keyword(s):  
Mouse Model ◽  
Peripheral Nerve ◽  
Peripheral Nerve System ◽  
Nerve System

scholarly journals Progressive Degeneration and Inhibition of Peripheral Nerve Regeneration in the SOD1-G93A Mouse Model of Amyotrophic Lateral Sclerosis

2018 ◽  
Vol 46 (6) ◽  
pp. 2358-2372 ◽  
Author(s):  
Binbin Deng ◽  
Wenjing Lv ◽  
Weisong Duan ◽  
Yakun Liu ◽  
Zhongyao Li ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Nervous System ◽  
Schwann Cell ◽  
Mouse Model ◽  
Peripheral Nerve ◽  
Peripheral Nervous System ◽  
Peripheral Nerves ◽  
Mutant Mice ◽  
Sod1 Mutant ◽  
Lateral Sclerosis

Background: Myelination, degeneration and regeneration are implicated in crucial responses to injury in the peripheral nervous system. Considering the progression of amyotrophic lateral sclerosis (ALS), we used the superoxide dismutase 1 (SOD1)-G93A transgenic mouse model of ALS to investigate the effects of mutant SOD1 on the peripheral nerves. Methods: Changes in peripheral nerve morphology were analyzed in SOD1 mutant mice at various stages of the disease by toluidine blue staining and electron microscopy (EM). Schwann cell proliferation and recruitment of inflammatory factors were detected by immunofluorescence staining and quantitative reverse transcription PCR and were compared between SOD1 mutant mice and control mice. Furthermore, western blotting (WB) and TUNEL staining were used to investigate axonal damage and Schwann cell survival in the sciatic nerves of mice in both groups. Results: An analysis of the peripheral nervous system in SOD1-G93A mice revealed the following novel features: (i) Schwann cells and axons in mutant mice underwent changes that were similar to those seen in the control mice during the early development of peripheral nerves. (ii) The peripheral nerves of SOD1-G93A mice developed progressive neuropathy, which presented as defects in axons and myelin, leading to difficulty in walking and reduced locomotor capacity at a late stage of the disease. (iii) Macrophages were recruited and accumulated, and nerve injury and a deficit in the blood-nerve barrier were observed. (iv) Proliferation and the inflammatory micro-environment were inhibited, which impaired the regeneration and remyelination of axons after crush injury in the SOD1-G93A mice. Conclusions: The mutant human SOD1 protein induced axonal and myelin degeneration during the progression of ALS and participated in axon remyelination and regeneration in response to injury.

scholarly journals Human Hair Derived Keratins Mediate Schwann Cell Behavior in vitro and Facilitate Rapid Peripheral Nerve Regeneration in vivo

2007 ◽  
Vol 21 (6) ◽  
Author(s):  
Paulina Sierpinski ◽  
Jeffrey Garrett ◽  
Jianjun Ma ◽  
Peter Apel ◽  
Tom Smith ◽  
...  
Keyword(s):  
Schwann Cell ◽  
Peripheral Nerve ◽  
Nerve Regeneration ◽  
Human Hair ◽  
Peripheral Nerve Regeneration ◽  
Cell Behavior

scholarly journals Use of a new mouse schwannoma tumour model to monitor changes in peripheral nerve morphology in Merlin null Schwann cells

2021 ◽  
Vol 23 (Supplement_4) ◽  
pp. iv7-iv8
Author(s):  
Marie Srotyr ◽  
Liyam Laraba ◽  
Glenn M Harper ◽  
Charlotte Lespade ◽  
Evyn Woodhouse ◽  
...  
Keyword(s):  
Nervous System ◽  
Sciatic Nerve ◽  
Schwann Cell ◽  
Peripheral Nerve ◽  
Schwann Cells ◽  
Erk Pathway ◽  
Tumour Formation ◽  
Immuno Histochemistry ◽  
Sciatic Nerves ◽  
Myelinating Schwann Cell

Abstract Aims Our lab is interested in signals that trigger schwannoma tumour formation and we have previously shown that peripheral nerve injury triggers tumour formation in nerves with Schwann cell-specific loss of the Merlin (NF2) tumour suppressor. The Ras/Raf/MAPK/ERK pathway activity in myelinating Schwann cells is involved in nerve regeneration, causing demyelination and recruitment of inflammatory cells in areas of nerve damage, as well as dedifferentiation of myelinating Schwann cells into a repair-competent state. We have used a mouse model expressing a tamoxifen-inducible Raf-Kinase estrogen receptor fusion protein (Raf-TR) in myelinating Schwann cells of the PNS in either a control wild-type Merlin or Merlin-null background. This allows us to determine the effects of an injury-like signal in Schwann cells and its role in generating schwannoma tumour development. We present here a detailed analysis of the proliferation of Schwann cells within the nerve and morphological changes in PNS structure following Raf-TR activation. Method The P0-promotor driving the Raf-TR transgene is active in myelinating Schwann cells but inactive in the non-myelinating population, allowing specific targeting of the myelinating Schwann cell population. In addition to the Raf-TR gene, the mice exhibit a separate P0-promotor controlled Cre floxed NF2 gene which undergoes Cre-mediated recombinase at embryonic day 13.5 causing NF2 knockout in all developing Schwann cells. Mice aged between 4-6 weeks received intraperitoneal injections of either 2mg Tamoxifen or oil vehicle for 5 consecutive days and were then studied at either 10 or 21 days post-first injection. The peripheral nervous system of the mice was studied with fluorescent immuno-histochemistry staining, semithin sections and transmission electron microscopy (TEM) on sciatic nerves and dorsal root ganglia (DRG). Results Activation of the Ras/Raf/MAPK/ERK pathway in NF2 null Schwann cells led to higher rates of proliferation within sciatic nerves at 10d post-tamoxifen injections. At both 10d and 21d Raf-TR+ NF2-null mice sciatic nerve fascicles were visibly larger with significantly more cell bodies present than controls, however at 21d the rate of proliferation had reduced. In the DRG, proliferation was higher in Raf-TR+ NF2-null mice compared to controls, with proliferation remaining high at 21 days. Quantitative imaging of peripheral nerve semi-thins analysed to date showed no significant difference in the number of myelin rings present in the fascicles between different genotypes. Additionally, dual immuno-histochemistry staining with Myelin Basic Protein and EdU, markers for myelin and proliferation respectively, appeared to show proliferation in the non-myelinating Schwann cell population. Results from staining with other cell markers will also be presented, as well as a detailed analysis of nerve structure using TEM. Conclusion While developmental myelination of Merlin-null Schwann cells appears largely normal, the reaction of Merlin-null Schwann cells in the nerve to an injury signal (activation of the Raf-TR) is remarkably different from those of control nerves. The high levels of proliferation in Merlin-null Schwann cells may be indicative of a higher tumorigenesis potential. While the proliferation of Merlin-null cells does reduce over time in the sciatic nerve, further experiments are now testing whether there may be ongoing tumour growth at other locations in the nervous system that are associated with NF2 tumours in human patients.

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