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https://www.readbyqxmd.com/read/28637335/astrocyte-produced-mir-146a-as-a-mediator-of-motor-neuron-loss-in-spinal-muscular-atrophy
#1
Samantha L Sison, Teresa N Patitucci, Emily R Seminary, Eric Villalon, Christian L Lorson, Allison D Ebert
Spinal muscular atrophy (SMA), the leading genetic cause of infant mortality, is caused by loss of the survival motor neuron-1 (SMN1) gene, which leads to motor neuron loss, muscle atrophy, respiratory distress, and death. Motor neurons exhibit the most profound loss, but the mechanisms underlying disease pathogenesis are not fully understood. Recent evidence suggests that motor neuron extrinsic influences, such as those arising from astrocytes, contribute to motor neuron malfunction and loss. Here we investigated both loss-of-function and toxic gain-of-function astrocyte mechanisms that could play a role in SMA pathology...
June 15, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28635301/stem-cell-mediated-paracrine-signaling-alters-fibroplasia-in-human-vocal-fold-fibroblasts-in-vitro
#2
Nao Hiwatashi, Renjie Bing, Iv Kraja, Ryan C Branski
OBJECTIVES: Interactions between mesenchymal stem cells (MSCs) and native vocal fold fibroblasts (VFFs) have not been described in spite of promising preliminary data regarding the effects of MSCs on vocal fold repair in vivo. The current study employed a conditioned media (CM) model to investigate the paracrine effects of bone marrow-derived mesenchymal stem cells (BMSCs) on VFFs. METHODS: Human VFFs were treated with transforming growth factor-β1 (TGF-β1; 10 ng/mL), CM from human BMSCs following 48 hours of TGF-β1 stimulation, or CM+TGF-β1...
June 1, 2017: Annals of Otology, Rhinology, and Laryngology
https://www.readbyqxmd.com/read/28634270/making-muscle-skeletal-myogenesis-in-vivo-and-in-vitro
#3
REVIEW
Jérome Chal, Olivier Pourquié
Skeletal muscle is the largest tissue in the body and loss of its function or its regenerative properties results in debilitating musculoskeletal disorders. Understanding the mechanisms that drive skeletal muscle formation will not only help to unravel the molecular basis of skeletal muscle diseases, but also provide a roadmap for recapitulating skeletal myogenesis in vitro from pluripotent stem cells (PSCs). PSCs have become an important tool for probing developmental questions, while differentiated cell types allow the development of novel therapeutic strategies...
June 15, 2017: Development
https://www.readbyqxmd.com/read/28632647/high-osteogenic-potential-of-adipose-and-muscle-derived-mesenchymal-stem-cells-in-spinal-ossification-model-mice
#4
Xizhe Liu, Gentaro Kumagai, Kanichiro Wada, Toshihiro Tanaka, Toru Asari, Kazuki Oishi, Taku Fujita, Hiroki Mizukami, Ken-Ichi Furukawa, Yasuyuki Ishibashi
STUDY DESIGN: Basic experiments in a mouse model of ossification of the posterior longitudinal ligament (OPLL). OBJECTIVE: To assess the osteogenic potential of mesenchymal stem cells (MSCs) obtained from muscle and adipose tissue in Tiptoe-walking (ttw) mice, in which cervical OPLL compresses the spinal cord and causes motor and sensory dysfunction. SUMMARY OF BACKGROUND DATA: In humans, MSCs have been implicated in the pathogenesis of cervical OPLL...
June 19, 2017: Spine
https://www.readbyqxmd.com/read/28631758/bioengineered-constructs-combined-with-exercise-enhance-stem-cell-mediated-treatment-of-volumetric-muscle-loss
#5
Marco Quarta, Melinda Cromie, Robert Chacon, Justin Blonigan, Victor Garcia, Igor Akimenko, Mark Hamer, Patrick Paine, Merel Stok, Joseph B Shrager, Thomas A Rando
Volumetric muscle loss (VML) is associated with loss of skeletal muscle function, and current treatments show limited efficacy. Here we show that bioconstructs suffused with genetically-labelled muscle stem cells (MuSCs) and other muscle resident cells (MRCs) are effective to treat VML injuries in mice. Imaging of bioconstructs implanted in damaged muscles indicates MuSCs survival and growth, and ex vivo analyses show force restoration of treated muscles. Histological analysis highlights myofibre formation, neovascularisation, but insufficient innervation...
June 20, 2017: Nature Communications
https://www.readbyqxmd.com/read/28630355/apical-and-basal-epitheliomuscular-f-actin-dynamics-during-hydra-bud-evagination
#6
Roland Aufschnaiter, Roland Wedlich-Söldner, Xiaoming Zhang, Bert Hobmayer
Bending of 2D cell sheets is a fundamental morphogenetic mechanism during animal development and reproduction. A critical player driving cell shape during tissue bending is the actin cytoskeleton. Much of our current knowledge about actin dynamics in whole organisms stems from studies of embryonic development in bilaterian model organisms. Here, we have analysed actin-based processes during asexual bud evagination in the simple metazoan Hydra We created transgenic Hydra strains stably expressing the actin marker Lifeact-GFP in either ectodermal or endodermal epitheliomuscular cells...
June 19, 2017: Biology Open
https://www.readbyqxmd.com/read/28630021/thyroid-hormone-signaling-and-deiodinase-actions-in-muscle-stem-progenitor-cells
#7
REVIEW
Raffaele Ambrosio, Maria Angela De Stefano, Daniela Di Girolamo, Domenico Salvatore
Thyroid hormone (TH) regulates such crucial biological functions as normal growth, development and metabolism of nearly all vertebrate tissues. In skeletal muscle, TH plays a critical role in regulating the function of satellite cells, the bona fide skeletal muscle stem cells. Deiodinases (D2 and D3) have been found to modulate the expression of various TH target genes in satellite cells. Regulation of the expression and activity of the deiodinases constitutes a cell-autonomous, pre-receptor mechanism that controls crucial steps during the various phases of myogenesis...
June 16, 2017: Molecular and Cellular Endocrinology
https://www.readbyqxmd.com/read/28628273/exposure-of-induced-pluripotent-stem-cell-derived-vascular-endothelial-and-smooth-muscle-cells-in-coculture-to-hemodynamics-induces-primary-vascular-cell-like-phenotypes
#8
Maria S Collado, Banumathi K Cole, Robert A Figler, Mark Lawson, David Manka, Michael B Simmers, Steve Hoang, Felipe Serrano, Brett R Blackman, Sanjay Sinha, Brian R Wamhoff
Human induced pluripotent stem cells (iPSCs) can be differentiated into vascular endothelial (iEC) and smooth muscle (iSMC) cells. However, because iECs and iSMCs are not derived from an intact blood vessel, they represent an immature phenotype. Hemodynamics and heterotypic cell:cell communication play important roles in vascular cell phenotypic modulation. Here we tested the hypothesis that hemodynamic exposure of iECs in coculture with iSMCs induces an in vivo-like phenotype. iECs and iSMCs were cocultured under vascular region-specific blood flow hemodynamics, and compared to hemodynamic cocultures of blood vessel-derived endothelial (pEC) and smooth muscle (pSMC) cells...
June 19, 2017: Stem Cells Translational Medicine
https://www.readbyqxmd.com/read/28627746/hepatic-perivascular-mesenchymal-stem-cells-with-myogenic-properties
#9
Sudheer Shenoy P, Bipasha Bose
Pericytes are multipotent mesenchymal stem cells (MSCs) located on the walls of blood vessels in various organs and are characterized as CD146(+) cells.. In this study, we have first immunohistochemically detected such pericytes in the perivascular regions of liver from two mouse genotypes namely wild-type (WT) and myostatin null (Mstn(-/-) ). We further isolated such pericytes using sorting as CD146(+) CD34(-) CD56(-) CD45(-) cells. The main finding of this study involve the contrasting -fibrogenic versus myogenic behavior of liver pericytes from WTMstn(-/-) ) mouse respectively...
June 19, 2017: Journal of Tissue Engineering and Regenerative Medicine
https://www.readbyqxmd.com/read/28627603/role-of-stem-cell-factor-in-the-regulation-of-icc-proliferation-and-detrusor-contraction-in-rats-with-an-underactive-bladder
#10
Jianli Feng, Jin Gao, Shan Zhou, Yuanfeng Liu, Yu Zhong, Yong Shu, Ming Sen Meng, Jiaqiang Yan, Danning Sun, Qiang Fang, Daodong Sun
Stem cell factor (SCF) is critical in regulating the proliferation, differentiation and function of the interstitial cells of Cajal (ICCs), which are closely associated with smooth muscle dysfunction. The present study aimed to examine the effect of SCF on ICC proliferation and detrusor contraction in rats with an underactive bladder. Sprague‑Dawley rats were divided into four groups comprising control, control+SCF, detrusor underactivity (DU), and DU+SCF groups. The ICC count was determined using immunofluorescence; serum levels of SCF were determined using an enzyme‑linked immunosorbent assay; mRNA and protein levels of c‑kit and SCF in tissues were assessed using reverse transcription‑quantitative polymerase chain reaction and western blot analyses, respectively...
June 9, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28624206/crispr-cas9-mediated-genome-editing-corrects-dystrophin-mutation-in-skeletal-muscle-stem-cells-in-a-mouse-model-of-muscle-dystrophy
#11
Pei Zhu, Furen Wu, Jeffrey Mosenson, Hongmei Zhang, Tong-Chuan He, Wen-Shu Wu
Muscle stem cells (MuSCs) hold great therapeutic potential for muscle genetic disorders, such as Duchenne muscular dystrophy (DMD). The CRISP/Cas9-based genome editing is a promising technology for correcting genetic alterations in mutant genes. In this study, we used fibrin-gel culture system to selectively expand MuSCs from crude skeletal muscle cells of mdx mice, a mouse model of DMD. By CRISP/Cas9-based genome editing, we corrected the dystrophin mutation in expanded MuSCs and restored the skeletal muscle dystrophin expression upon transplantation in mdx mice...
June 16, 2017: Molecular Therapy. Nucleic Acids
https://www.readbyqxmd.com/read/28624186/gaa-deficiency-in-pompe-disease-is-alleviated-by-exon-inclusion-in-ipsc-derived-skeletal-muscle-cells
#12
Erik van der Wal, Atze J Bergsma, Tom J M van Gestel, Stijn L M In 't Groen, Holm Zaehres, Marcos J Araúzo-Bravo, Hans R Schöler, Ans T van der Ploeg, W W M Pim Pijnappel
Pompe disease is a metabolic myopathy caused by deficiency of the acid α-glucosidase (GAA) enzyme and results in progressive wasting of skeletal muscle cells. The c.-32-13T>G (IVS1) GAA variant promotes exon 2 skipping during pre-mRNA splicing and is the most common variant for the childhood/adult disease form. We previously identified antisense oligonucleotides (AONs) that promoted GAA exon 2 inclusion in patient-derived fibroblasts. It was unknown how these AONs would affect GAA splicing in skeletal muscle cells...
June 16, 2017: Molecular Therapy. Nucleic Acids
https://www.readbyqxmd.com/read/28623422/effects-of-omega-3-on-matrix-metalloproteinase-9-myoblast-transplantation-and-satellite-cell-activation-in-dystrophin-deficient-muscle-fibers
#13
Samara Camaçari de Carvalho, Sajedah M Hindi, Ashok Kumar, Maria Julia Marques
In Duchenne muscular dystrophy (DMD), lack of dystrophin leads to progressive muscle degeneration, with DMD patients suffering from cardiorespiratory failure. Cell therapy is an alternative to life-long corticoid therapy. Satellite cells, the stem cells of skeletal muscles, do not completely compensate for the muscle damage in dystrophic muscles. Elevated levels of proinflammatory and profibrotic factors, such as metalloproteinase 9 (MMP-9), impair muscle regeneration, leading to extensive fibrosis and poor results with myoblast transplantation therapies...
June 17, 2017: Cell and Tissue Research
https://www.readbyqxmd.com/read/28621200/muscle-derived-stem-cells-in-peripheral-nerve-regeneration-reality-or-illusion
#14
Jing Zhou, Haiyan Cui, Haibin Lu, Zhuqiu Xu, Weifeng Feng, Lulu Chen, Xiaolei Jin, Xiaonan Yang, Zuoliang Qi
Owing to the complicated and time-consuming regenerative process, the repair of injured peripheral nerves depends largely on ongoing stem-cell therapy. Decades ago, researchers successfully isolated and identified muscle-derived stem cells (MDSCs) and discovered their potential for multidifferentiation. MDSCs play an important role in trauma repair associated with neuromuscular and vascular injury by simultaneously promoting tissue regrowth via direct differentiation and systematic secretion under physiological conditions...
June 16, 2017: Regenerative Medicine
https://www.readbyqxmd.com/read/28616907/-differentiation-of-cultivated-cd45-cd31-mouse-lung-side-population-cells-into-vascular-smooth-muscle-cells-in-vitro
#15
Ming-Jie Zhang, Zong-Ze Li, Yang Xu, Simon Xun Liang
OBJECTIVES: To investigate the characteristics of differentiation of lung side population cells (LSP cells)in vitro. METHODS: CD45(-)/CD31(+) LSP cells sorted by flow cytometry were taken from mouse lung tissues and cultured for 14 d. The cultured LSP cells were observed with colony formation assay and flow cytometryin vitro. The mRNA expressions of ATP-binding cassette transporter G2 (ABCG2), smooth muscle actin (SMA) and α-smooth muscle tropomyosin (α-SMT) in both freshly isolated LSP cells and cultured LSP cells were examined...
May 2017: Sichuan da Xue Xue Bao. Yi Xue Ban, Journal of Sichuan University. Medical Science Edition
https://www.readbyqxmd.com/read/28616376/genome-editing-and-muscle-stem-cells-as-a-therapeutic-tool-for-muscular-dystrophies
#16
REVIEW
Veronica Pini, Jennifer E Morgan, Francesco Muntoni, Helen C O'Neill
PURPOSE OF REVIEW: Muscular dystrophies are a group of severe degenerative disorders characterized by muscle fiber degeneration and death. Therapies designed to restore muscle homeostasis and to replace dying fibers are being experimented, but none of those in clinical trials are suitable to permanently address individual gene mutation. The purpose of this review is to discuss genome editing tools such as CRISPR/Cas (clustered regularly interspaced short palindromic repeats/CRISPR-associated), which enable direct sequence alteration and could potentially be adopted to correct the genetic defect leading to muscle impairment...
2017: Current Stem Cell Reports
https://www.readbyqxmd.com/read/28615691/human-myogenic-reserve-cells-are-quiescent-stem-cells-that-contribute-to-muscle-regeneration-after-intramuscular-transplantation-in-immunodeficient-mice
#17
Thomas Laumonier, Flavien Bermont, Pierre Hoffmeyer, Vincent Kindler, Jacques Menetrey
Satellite cells, localized within muscles in vivo, are Pax7(+) muscle stem cells supporting skeletal muscle growth and regeneration. Unfortunately, their amplification in vitro, required for their therapeutic use, is associated with reduced regenerative potential. In the present study, we investigated if human myogenic reserve cells (MRC) obtained in vitro, represented a reliable cell source for muscle repair. For this purpose, primary human myoblasts were freshly isolated and expanded. After 2 days of differentiation, 62 ± 2...
June 14, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28615630/non-muscle-myosin-ii-is-required-for-correct-fate-specification-in-the-caenorhabditis-elegans-seam-cell-divisions
#18
Siyu Serena Ding, Alison Woollard
During development, cell division often generates two daughters with different developmental fates. Distinct daughter identities can result from the physical polarity and size asymmetry itself, as well as the subsequent activation of distinct fate programmes in each daughter. Asymmetric divisions are a feature of the C. elegans seam lineage, in which a series of post-embryonic, stem-like asymmetric divisions give rise to an anterior daughter that differentiates and a posterior daughter that continues to divide...
June 14, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28614918/the-significance-of-sdf-1alpha-cxcr4-axis-in-in-vivo-angiogenic-ability-of-human-periodontal-ligament-stem-cells
#19
Yoon-Kyung Bae, Gee-Hye Kim, Jae Cheoun Lee, Byoung-Moo Seo, Kyeung-Min Joo, Gene Lee, Hyun Nam
Periodontal ligament stem cells (PDLSCs) are multipotent stem cells derived from periodontium and have mesenchymal stem cell (MSC)-like characteristics. Recently, the perivascular region was recognized as the developmental origin of MSCs, which suggests the in vivo angiogenic potential of PDLSCs. In this study, we investigated whether PDLSCs could be a potential source of perivascular cells, which could contribute to in vivo angiogenesis. PDLSCs exhibited typical MSC-like characteristics such as the expression pattern of surface markers (CD29, CD44, CD73, and CD105) and differentiation potentials (osteogenic and adipogenic differentiation)...
June 14, 2017: Molecules and Cells
https://www.readbyqxmd.com/read/28614716/the-notch1-snail1-mef2c-pathway-regulates-growth-and-self-renewal-in-embryonal-rhabdomyosarcoma
#20
Myron S Ignatius, Madeline N Hayes, Riadh Lobbardi, Eleanor Y Chen, Karin M McCarthy, Prethish Sreenivas, Zainab Motala, Adam D Durbin, Aleksey Molodtsov, Sophia Reeder, Alexander Jin, Sivasish Sindiri, Brian C Beleyea, Deepak Bhere, Matthew S Alexander, Khalid Shah, Charles Keller, Corinne M Linardic, Petur G Nielsen, David Malkin, Javed Khan, David M Langenau
Tumor-propagating cells (TPCs) share self-renewal properties with normal stem cells and drive continued tumor growth. However, mechanisms regulating TPC self-renewal are largely unknown, especially in embryonal rhabdomyosarcoma (ERMS)-a common pediatric cancer of muscle. Here, we used a zebrafish transgenic model of ERMS to identify a role for intracellular NOTCH1 (ICN1) in increasing TPCs by 23-fold. ICN1 expanded TPCs by enabling the de-differentiation of zebrafish ERMS cells into self-renewing myf5+ TPCs, breaking the rigid differentiation hierarchies reported in normal muscle...
June 13, 2017: Cell Reports
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