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https://www.readbyqxmd.com/read/29910711/functional-consequences-of-the-postnatal-switch-from-neonatal-to-mutant-adult-glycine-receptor-%C3%AE-1-subunits-in-the-shaky-mouse-model-of-startle-disease
#1
Natascha Schaefer, Fang Zheng, Johannes van Brederode, Alexandra Berger, Sophie Leacock, Hiromi Hirata, Christopher J Paige, Robert J Harvey, Christian Alzheimer, Carmen Villmann
Mutations in GlyR α1 or β subunit genes in humans and rodents lead to severe startle disease characterized by rigidity, massive stiffness and excessive startle responses upon unexpected tactile or acoustic stimuli. The recently characterized startle disease mouse mutant shaky carries a missense mutation (Q177K) in the β8-β9 loop within the large extracellular N-terminal domain of the GlyR α1 subunit. This results in a disrupted hydrogen bond network around K177 and faster GlyR decay times. Symptoms in mice start at postnatal day 14 and increase until premature death of homozygous shaky mice around 4-6 weeks after birth...
2018: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/29910095/sporadic-late-onset-nemaline-myopathy-with-monoclonal-gammopathy-of-undetermined-significance-slonm-mgus-an-alternative-treatment-using-cyclophosphamide-thalidomide-dexamethasone-ctd-regimen
#2
Theerawat Kumutpongpanich, Weerapat Owattanapanich, Jantima Tanboon, Ichizo Nishino, Kanokwan Boonyapisit
Sporadic late-onset nemaline myopathy with monoclonal gammopathy of undetermined significance is a rare subacute adult-onset myopathy. Without appropriate treatment, the prognosis is unfavorable and can be fatal. Various efficacious treatment options have been reported. High dose melphalan followed by autologous stem cell transplantation is the most used option with favorable outcome. Nevertheless, potentially safer alternative regimens await exploration. Here, we report the case of sporadic late-onset nemaline myopathy with monoclonal gammopathy of undetermined significance in a 33-year-old man with significant clinical improvement and complete remission of monoclonal gammopathy after 5 cycles of cyclophosphamide, thalidomide, and dexamethasone regimen...
May 16, 2018: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/29908230/additional-cytogenetic-abnormalities-with-philadelphia-chromosome-positive-acute-lymphoblastic-leukemia-on-allogeneic-stem-cell-transplantation-in-the-tyrosine-kinase-inhibitor-era
#3
Yu Akahoshi, Shuichi Mizuta, Hiroaki Shimizu, Naoyuki Uchida, Takahiro Fukuda, Heiwa Kanamori, Makoto Onizuka, Yukiyasu Ozawa, Kazuteru Ohashi, Shuichi Ohta, Tetsuya Eto, Junji Tanaka, Yoshiko Atsuta, Shinichi Kako
Cytogenetic abnormalities are well-known and powerful independent prognostic factor for various hematological disorders. Although the combination of chemotherapy with tyrosine kinase inhibitor (TKI) is now considered the standard of care in patients with Philadelphia chromosome-positive acute lymphoblastic leukemia, little is known about the impact of additional cytogenetic abnormalities (ACAs). Therefore, we retrospectively evaluated 1375 adult patients who underwent their first allogeneic hematopoietic stem cell transplantation in the TKI era...
June 13, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29907642/telomerase-reverse-transcriptase-coordinates-with-the-epithelial-to-mesenchymal-transition-through-a-feedback-loop-to-define-properties-of-breast-cancer-stem-cells
#4
Ahmed El-Badawy, Nehal I Ghoneim, Mohamed A Nasr, Hoda Elkhenany, Toka A Ahmed, Sara M Ahmed, Nagwa El-Badri
Telomerase and its core component, telomerase reverse transcriptase (hTERT), are critical for stem cell compartment integrity. Normal adult stem cells have the longest telomeres in a given tissue, a property mediated by high hTERT expression and high telomerase enzymatic activity. In contrast, cancer stem cells (CSCs) have short telomeres despite high expression of hTERT, indicating that the role of hTERT in CSCs is not limited to telomere elongation and/or maintenance. The function of hTERT in CSCs remains poorly understood...
June 15, 2018: Biology Open
https://www.readbyqxmd.com/read/29907232/intramuscular-e-selectin-adeno-associated-virus-gene-therapy-promotes-wound-healing-in-an-ischemic-mouse-model
#5
Punam P Parikh, Roberta M Lassance-Soares, Hongwei Shao, Manuela M Regueiro, Yan Li, Zhao-Jun Liu, Omaida C Velazquez
BACKGROUND: Poor wound healing in critical limb ischemia (CLI) is attributed to impaired neovascularization and reperfusion. Optimizing the ischemic wound with adhesion molecules that enhance stem cell homing may revolutionize treatment. The purpose of this study is to test the efficacy of adhesion molecule E-selectin on wound healing in an ischemic mouse wound. METHODS: Adult FVB/NJ mice underwent unilateral femoral artery and vein ligation to induce CLI. A 4-mm punch biopsy wound was created on the anterior thigh to simulate ischemic wounds...
August 2018: Journal of Surgical Research
https://www.readbyqxmd.com/read/29907164/optimizing-the-method-for-generation-of-integration-free-induced-pluripotent-stem-cells-from-human-peripheral-blood
#6
Haihui Gu, Xia Huang, Jing Xu, Lili Song, Shuping Liu, Xiao-Bing Zhang, Weiping Yuan, Yanxin Li
BACKGROUND: Generation of induced pluripotent stem cells (iPSCs) from human peripheral blood provides a convenient and low-invasive way to obtain patient-specific iPSCs. The episomal vector is one of the best approaches for reprogramming somatic cells to pluripotent status because of its simplicity and affordability. However, the efficiency of episomal vector reprogramming of adult peripheral blood cells is relatively low compared with cord blood and bone marrow cells. METHODS: In the present study, integration-free human iPSCs derived from peripheral blood were established via episomal technology...
June 15, 2018: Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/29906446/prospectively-isolated-tetraspanin-neoblasts-are-adult-pluripotent-stem-cells-underlying-planaria-regeneration
#7
An Zeng, Hua Li, Longhua Guo, Xin Gao, Sean McKinney, Yongfu Wang, Zulin Yu, Jungeun Park, Craig Semerad, Eric Ross, Li-Chun Cheng, Erin Davies, Kai Lei, Wei Wang, Anoja Perera, Kate Hall, Allison Peak, Andrew Box, Alejandro Sánchez Alvarado
Proliferating cells known as neoblasts include pluripotent stem cells (PSCs) that sustain tissue homeostasis and regeneration of lost body parts in planarians. However, the lack of markers to prospectively identify and isolate these adult PSCs has significantly hampered their characterization. We used single-cell RNA sequencing (scRNA-seq) and single-cell transplantation to address this long-standing issue. Large-scale scRNA-seq of sorted neoblasts unveiled a novel subtype of neoblast (Nb2) characterized by high levels of PIWI-1 mRNA and protein and marked by a conserved cell-surface protein-coding gene, tetraspanin 1 (tspan-1)...
June 14, 2018: Cell
https://www.readbyqxmd.com/read/29905527/adult-zebrafish-langerhans-cells-arise-from-hematopoietic-stem-progenitor-cells
#8
Sicong He, Jiahao Chen, Yunyun Jiang, Yi Wu, Lu Zhu, Wan Jin, Changlong Zhao, Tao Yu, Tienan Wang, Shuting Wu, Xi Lin, Jianan Y Qu, Zilong Wen, Wenqing Zhang, Jin Xu
The origin of Langerhans cells (LCs), which are skin epidermis-resident macrophages, remains unclear. Current lineage tracing of LCs largely relies on the promoter-Cre-LoxP system, which often gives rise to contradictory conclusions with different promoters. Thus, reinvestigation with an improved tracing method is necessary. Here, using a laser-mediated temporal-spatial resolved cell labeling method, we demonstrated that most adult LCs originated from the ventral wall of the dorsal aorta (VDA), an equivalent to the mouse aorta, gonads, and mesonephros (AGM), where both hematopoietic stem cells (HSCs) and non-HSC progenitors are generated...
June 15, 2018: ELife
https://www.readbyqxmd.com/read/29905460/identification-of-compounds-that-decrease-glioblastoma-growth-and-glucose-uptake-in-vitro
#9
Catherine J Libby, Sixue Zhang, Gloria A Benavides, Sarah E Scott, Yanjie Li, Matthew Redmann, Anh Nhat Tran, Arphaxad Otamias, Victor Darley-Usmar, Marek Napierala, Jianhua Zhang, Corinne Elizabeth Augelli-Szafran, Wei Zhang, Anita B Hjelmeland
Tumor heterogeneity has hampered the development of novel effective therapeutic options for aggressive cancers, including the deadly primary adult brain tumor glioblastoma (GBM). Intratumoral heterogeneity is partially attributed to the tumor initiating cell (TIC) subset that contains highly tumorigenic, stem-like cells. TICs display metabolic plasticity but can have a reliance on aerobic glycolysis. Elevated expression of GLUT1 and GLUT3 is present in many cancer types, with GLUT3 being preferentially expressed in brain TICs (BTICs) to increase survival in low nutrient tumor microenvironments, leading to tumor maintenance...
June 15, 2018: ACS Chemical Biology
https://www.readbyqxmd.com/read/29905055/-effect-of-chitosan-porous-scaffolds-combined-with-bone-marrow-mesenchymal-stem-cells-in-repair-of-neurological-deficit-after-traumatic-brain-injury-in-rats
#10
Keke Tan, Xiuxiu Wang, Jun Zhang, Zhigang Zhuang, Tieli Dong
Objective: To investigate the possibility and effect of chitosan porous scaffolds combined with bone marrow mesenchymal stem cells (BMSCs) in repair of neurological deficit after traumatic brain injury (TBI) in rats. Methods: BMSCs were isolated, cultured, and passaged by the method of bone marrow adherent culture. The 3rd generation BMSCs were identified by the CD29 and CD45 surface antigens and marked by 5-bromo-2-deoxyuridine (BrdU). The chitosan porous scaffolds were produced by the method of freeze-drying...
June 1, 2018: Chinese Journal of Reparative and Reconstructive Surgery
https://www.readbyqxmd.com/read/29905054/-research-of-acellular-xenogeneic-nerve-combined-with-adipose-derived-stem-cells-and-platelet-rich-plasma-in-repair-of-rabbit-facial-nerve-injury
#11
Yanna Sun, Rongming Zhang, Xu Mao, Mengshu Zhang
Objective: To investigate the early effects of acellular xenogeneic nerve combined with adipose-derived stem cells (ADSCs) and platelet rich plasma (PRP) in repairing facial nerve injury in rabbits. Methods: The bilateral sciatic nerves of 15 3-month-old male Sprague-Dawley rats were harvested and decellularized as xenografts. The allogeneic ADSCs were extracted from the neck and back fat pad of healthy adult New Zealand rabbits with a method of digestion by collagenase type Ⅰ and the autologous PRP was prepared by two step centrifugation...
June 1, 2018: Chinese Journal of Reparative and Reconstructive Surgery
https://www.readbyqxmd.com/read/29904629/temporal-changes-in-nucleus-morphology-lamin-a-c-and-histone-methylation-during-nanotopography-induced-neuronal-differentiation-of-stem-cells
#12
Soneela Ankam, Benjamin K K Teo, Grace Pohan, Shawn W L Ho, Choon K Lim, Evelyn K F Yim
Stem cell differentiation can be regulated by biophysical cues such as nanotopography. It involves sensing and integration of these biophysical cues into their transcriptome with a mechanism that is yet to be discovered. In addition to the cytoskeletal and focal adhesion remodeling, nanotopography has also been shown to modulate nucleus morphology. Here, we studied the effect of nanotopography on the temporal changes in nuclei of human embryonic stem cells (hESCs) and human mesenchymal stem cells (hMSCs). Using a high throughput Multi-architecture (MARC) chip analysis, the circularity of the stem cell nuclei changed significantly on different patterns...
2018: Frontiers in Bioengineering and Biotechnology
https://www.readbyqxmd.com/read/29904155/moringin-induces-neural-differentiation-in-the-stem-cell-of-the-human-periodontal-ligament
#13
Letizia Romeo, Francesca Diomede, Agnese Gugliandolo, Domenico Scionti, Fabrizio Lo Giudice, Veronica Lanza Cariccio, Renato Iori, Placido Bramanti, Oriana Trubiani, Emanuela Mazzon
The therapeutic strategies for neurodegenerative diseases still represent a vast research field because of the lack of targeted, effective and resolutive treatment for neurodegenerative diseases. The use of stem cell-based therapy is an alternative approach that could lead to the replacement of damaged neuronal tissue. For this purpose, adult mesenchymal stem cells (MSC), including periodontal ligament stem cells (PDLSCs), could be very useful for their differentiation capacity, easy isolation and the ability to perform an autologous implant...
June 14, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29903864/spatio-temporal-neural-stem-cell-behavior-that-leads-to-both-perfect-and-imperfect-structural-brain-regeneration-in-adult-newts
#14
Yuko Urata, Wataru Yamashita, Takeshi Inoue, Kiyokazu Agata
Adult newts can regenerate large parts of their brain from adult neural stem cells (NSCs), but how adult NSCs reorganize brain structures during regeneration remains unclear. In development, elaborate brain structures are produced under broadly coordinated regulations of embryonic NSCs in the neural tube, whereas brain regeneration entails exquisite control of the reestablishment of certain brain parts, suggesting a yet-unknown mechanism directs NSCs upon partial brain excision. Here we report that upon one-quarter excision of the adult newt ( Pleurodeles waltl ) mesencephalon, active participation of local NSCs around specific brain subregions' boundaries leads to some imperfect and some perfect brain regeneration along an individual's rostrocaudal axis...
June 14, 2018: Biology Open
https://www.readbyqxmd.com/read/29902505/short-term-heat-exposure-promotes-hippocampal-neurogenesis-via-activation-of-angiotensin-ii-type-1-receptor-in-adult-rats
#15
Yuka Koyama, Takao Mukuda, Sawako Hamasaki, Hironobu Nakane, Toshiyuki Kaidoh
Angiotensin II (Ang II) synthesized in response to body fluid loss caused by actions such as sweating and breathing is today considered as one of the essential factors for promoting hippocamal neurogenesis. Because heat-stimuli, along with exercise, increases systemic levels of Ang II, the effects of short-term heat exposure on hippocampal neurogenesis were examined in adult male rats. When rats were exposed daily to a 1 h heat treatment (36.0 ± 0.1°C) during a 7 d experimental period, the number of doublecortin-immunoreactive newborn cells in the hippocampal dentate gyrus was increased approximately 1...
June 11, 2018: Neuroscience
https://www.readbyqxmd.com/read/29902500/the-role-of-neuro-epithelial-like-and-radial-glial-stem-and-progenitor-cells-in-development-plasticity-and-repair
#16
REVIEW
Benjamin W Lindsey, Zachary J Hall, Aurélie Heuzé, Jean-Stéphane Joly, Vincent Tropepe, Jan Kaslin
Neural stem and progenitor cells (NSPCs) are the primary source of new neurons in the brain and serve critical roles in tissue homeostasis and plasticity throughout life. Within the vertebrate brain, NSPCs are located within distinct neurogenic niches differing in their location, cellular composition, and proliferative behaviour. Heterogeneity in the NSPC population is hypothesized to reflect varying capacities for neurogenesis, plasticity and repair between different neurogenic zones. Since the discovery of adult neurogenesis, studies have predominantly focused on the behaviour and biological significance of adult NSPCs (aNSPCs) in rodents...
June 11, 2018: Progress in Neurobiology
https://www.readbyqxmd.com/read/29902485/a-nanoformulation-for-the-preferential-accumulation-in-adult-neurogenic-niches
#17
Catarina Praça, Akhilesh Rai, Tiago Santos, Ana C Cristovão, Sonia L Pinho, Romeo Cecchelli, Marie-Pierre Dehouck, Liliana Bernardino, Lino S Ferreira
Stimulation of adult neurogenesis by targeting the endogenous neural stem cells (NSCs), located in hippocampus and subventricular zone (SVZ), with nanoformulations has been proposed for brain repair in cases of neurodegenerative diseases. Unfortunately, it is relatively unknown the nanoformulation properties to facilitate their accumulation in the neurogenic niches after intravenous injection. Here, we have screened different gold-based formulations having variable morphology, surface chemistry and responsiveness to light for their capacity to cross the blood brain barrier (BBB) and accumulate preferentially in the neurogenic niches...
June 11, 2018: Journal of Controlled Release: Official Journal of the Controlled Release Society
https://www.readbyqxmd.com/read/29902110/celastrol-enhances-atoh1-expression-in-inner-ear-stem-cells-and-promotes-their-differentiation-into-functional-auditory-neuronal-like-cells
#18
Zhao Han, Yu-Yan Gu, Ning Cong, Rui Ma, Fang-Lu Chi
We aimed to investigate the beneficial effect of Celastrol on inner ear stem cells and potential therapeutic value for hearing loss. The inner ear stem cells were isolated and characterized from utricular sensory epithelium of adult mice. The stemness was evaluated by sphere formation assay. The relative expressions of Atoh1, MAP-2 and Myosin VI were measured by RT-PCR and immunoblotting. The up-regulation of MAP-2 was also analysed with immunofluorescence. The in vitro neuronal excitability was interrogated by calcium oscillation...
June 14, 2018: Organogenesis
https://www.readbyqxmd.com/read/29899574/pre-engraftment-cytomegalovirus-dnaemia-in-allogeneic-hematopoietic-stem-cell-transplant-recipients-incidence-risk-factors-and-clinical-outcomes
#19
Carlos Solano, Estela Giménez, Eliseo Albert, Eva María Mateo, Montserrat Gómez, Rosa Goterris, Ariadna Pérez, Paula Amat, Juan Carlos Hernández-Boluda, Marc Poch, José Luis Piñana, David Navarro
To gauge the risk of delaying initiation of prophylaxis with letermovir from the time of donor infusion to prevent CMV infection in allo-HSCT recipients we investigated the clinical outcomes of CMV DNAemia episodes occurring before engraftment, and compared to that of episodes developing after engraftment (up to day +365). A total of 197 consecutive adult patients were included. Plasma CMV DNA load was monitored by real-time PCR assays [limit of detection: 31 IU/ml]. A total of 150 out of 197 patients had CMV DNAemia (cumulative incidence of 77%; 95% CI, 73-81%), and 38 out of the 197 patients developed it before engraftment (cumulative incidence, 19%; 95% CI, 10-30...
June 13, 2018: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/29899571/the-safety-and-efficacy-of-clofarabine-in-combination-with-high-dose-cytarabine-and-total-body-irradiation-myeloablative-conditioning-and-allogeneic-stem-cell-transplantation-in-children-adolescents-and-young-adults-caya-with-poor-risk-acute-leukemia
#20
Jessica Hochberg, Stacey Zahler, Mark B Geyer, Nan Chen, Jennifer Krajewski, Lauren Harrison, Olga Militano, M Fevzi Ozkaynak, Alexandra C Cheerva, Julie Talano, Theodore B Moore, Alfred P Gillio, Mark C Walters, Lee Ann Baxter-Lowe, Carl Hamby, Mitchell S Cairo
Acute leukemias in children with CR3, refractory relapse, or induction failure (IF) have a poor prognosis. Clofarabine has single agent activity in relapsed leukemia and synergy with cytarabine. We sought to determine the safety and overall survival in a Phase I/II trial of conditioning with clofarabine (doses 40 - 52 mg/m2 ), cytarabine 1000 mg/m2 , and 1200 cGy TBI followed by alloSCT in children, adolescents, and young adults with poor-risk leukemia. Thirty-seven patients; Age 12 years (1-22 years); ALL/AML: 34:3 (18 IF, 10 CR3, 13 refractory relapse); 15 related, 22 unrelated donors...
June 13, 2018: Bone Marrow Transplantation
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