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https://www.readbyqxmd.com/read/29051055/skeletal-impairment-in-pierson-syndrome-is-there-a-role-for-laminin%C3%AE-2-in-bone-physiology
#1
Camille Beaufils, Delphine Farlay, Irma Machuca-Gayet, Alice Fassier, Martin Zenker, Caroline Freychet, Edith Bonnelye, Aurélia Bertholet-Thomas, Bruno Ranchin, Justine Bacchetta
INTRODUCTION: Pierson syndrome is caused by a mutation of LAMB2, encoding for laminin β2. Clinical phenotype is variable but usually associates congenital nephrotic syndrome (CNS) and ocular abnormalities. Neuromuscular impairment has also been described. METHODS: We report on a 15-year old girl, suffering from Pierson Syndrome, who developed severe bone deformations during puberty. This patient initially displayed CNS and microcoria, leading to the clinical diagnosis of Pierson syndrome...
October 16, 2017: Bone
https://www.readbyqxmd.com/read/29046806/development-of-a-fully-automated-chemiluminescence-immunoassay-for-urine-monomeric-laminin-%C3%AE-2-as-a-promising-diagnostic-tool-of-non-muscle-invasive-bladder-cancer
#2
Masatoshi Nakagawa, Takashi Karashima, Masayuki Kamada, Eisaku Yoshida, Toru Yoshimura, Masanori Nojima, Keiji Inoue, Taro Shuin, Motoharu Seiki, Naohiko Koshikawa
BACKGROUND: Monomeric laminin-γ2 in urine is a potential biomarker for bladder cancer. However, the current detection system uses an antibody that cannot discriminate between monomeric laminin-γ2 and the heterotrimeric γ2 chain of laminin-332, which may cause false-positive reactions. The present study aimed to develop a fully automated chemiluminescence immunoassay system using a specific monoclonal antibody against monomeric laminin-γ2. METHODS: In total, 237 urine specimens (84 from patients with bladder cancer, 48 from patients with benign urological disease, and 105 from healthy donors) were collected, and monomeric laminin-γ2 values in the urine were measured using a fully automated chemiluminescence immunoassay...
2017: Biomarker Research
https://www.readbyqxmd.com/read/29044418/friedreich-ataxia-developmental-failure-of-the-dorsal-root-entry-zone
#3
Arnulf H Koeppen, Alyssa B Becker, Jiang Qian, Benjamin B Gelman, Joseph E Mazurkiewicz
Dorsal root ganglia, dorsal roots (DR), and dorsal root entry zones (DREZ) are vulnerable to frataxin deficiency in Friedreich ataxia (FA). A previously unrecognized abnormality is the intrusion of astroglial tissue into DR. Segments of formalin-fixed upper lumbar spinal cord of 13 homozygous and 2 compound heterozygous FA patients were sectioned longitudinally to represent DREZ and stained for glial fibrillary acidic protein (GFAP), S100, vimentin, the central nervous system (CNS)-specific myelin protein proteolipid protein, the peripheral nervous system (PNS) myelin proteins PMP-22 and P0, and the Schwann cell proteins laminin, alpha-dystroglycan, and periaxin...
November 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/29044412/correlation-between-extravasation-and-alterations-of-cerebrovascular-laminin-and-%C3%AE-dystroglycan-immunoreactivity-following-cryogenic-lesions-in-rats
#4
Mihály Kálmán, László Tóth, Dávid Szöllosi, Erzsébet Oszwald, Judit Mahalek, Sam Sadeghian
The blood-brain barrier becomes "leaky" following lesions. Former studies revealed that following lesions the immunoreactivity of cerebrovascular laminin becomes detectable whereas that of β-dystroglycan disappears. These alterations may be indicators of glio-vascular decoupling that may result in the impairment of the blood-brain-barrier. This study investigates correlation between the post-lesion extravasation and the above-mentioned immunohistochemical alterations. Following cryogenic lesions, the survival periods lasted 5, 10, 30 minutes, 1 or 12 hours, or 1 day...
November 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/29039438/chondroitin-sulfate-proteoglycans-inhibit-the-migration-and-differentiation-of-oligodendrocyte-precursor-cells-and-its-counteractive-interaction-with-laminin
#5
Yi Sun, Yingping Deng, Mili Xiao, Lan Hu, Zhihua Li, Chao Chen
Cerebral white matter injury (WMI) is a recognized problem in premature infants, particularly in those with very low birth weights. Although the survival rate of premature infants has notably increased due to the advancement of modern medical treatments, their likelihood of developmental disability is higher than infants with an average birth weight. It has been previously reported that oligodendrocyte precursor cells (OPCs) are selectively vulnerable to WMI in premature infants. Following brain injury, glial scars may develop within the white matter...
September 27, 2017: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/29037855/nephrosphere-derived-cells-are-induced-to-multilineage-differentiation-when-cultured-on-human-decellularized-kidney-scaffolds
#6
Silvia Bombelli, Chiara Meregalli, Carla Scalia, Giorgio Bovo, Barbara Torsello, Sofia De Marco, Massimiliano Cadamuro, Paolo Viganò, Guido Strada, Giorgio Cattoretti, Cristina Bianchi, Roberto A Perego
In end-stage chronic kidney disease, the option of organ transplantation is limited due to the scarce availability of kidneys. The combination of stem cell research, regenerative medicine, and tissue engineering seems a promising approach to produce new transplantable kidneys. Currently, the possibility to repopulate naturally obtained scaffolds with cells of different sources is advancing. Our aim was to test, for the first time, whether the nephrosphere (NS) cells, composed by renal stem/progenitor-like cells, were able to repopulate different nephron portions of renal extracellular matrix scaffolds obtained after decellularization of human renal tissue slices...
October 13, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/29036200/the-effect-of-the-pathological-v72i-d109n-and-t190m-missense-mutations-on-the-molecular-structure-of-%C3%AE-dystroglycan
#7
Sonia Covaceuszach, Manuela Bozzi, Maria Giulia Bigotti, Francesca Sciandra, Petr V Konarev, Andrea Brancaccio, Alberto Cassetta
Dystroglycan (DG) is a highly glycosylated protein complex that links the cytoskeleton with the extracellular matrix, mediating fundamental physiological functions such as mechanical stability of tissues, matrix organization and cell polarity. A crucial role in the glycosylation of the DG α subunit is played by its own N-terminal region that is required by the glycosyltransferase LARGE. Alteration in this O-glycosylation deeply impairs the high affinity binding to other extracellular matrix proteins such as laminins...
2017: PloS One
https://www.readbyqxmd.com/read/29034805/in-vitro-investigation-of-the-effect-of-matrix-molecules-on-the-behavior-of-colon-cancer-cells-under-the-effect-of-geldanamycin-derivative
#8
Kamil Vural, Funda Kosova, Feyzan Özdal Kurt, İbrahim Tuğlu
The chaperone-binding drug, 17-allylamino-17-demethoxygeldanamycin, has recently come into clinical use. It is a derivative of geldanamycin, an ansamycin benzoquinone antibiotic with anti-carcinogenic effect. Understanding the effect of this drug on the cancer cells and their niche is important for treatment. We applied 17-allylamino-17-demethoxygeldanamycin to colon cancer cell line (Colo 205) on matrix molecules to investigate the relationship of apoptosis with terminal deoxynucleotidyl transferase dUTP nick end labeling immunocytochemistry and related gene expression...
October 2017: Tumour Biology: the Journal of the International Society for Oncodevelopmental Biology and Medicine
https://www.readbyqxmd.com/read/29023785/cns-synapses-are-stabilized-trans-synaptically-by-laminins-and-laminin-interacting-proteins
#9
Dale D Hunter, Mary K Manglapus, Galina Bachay, Thomas Claudepierre, Michael W Dolan, Kelly-Ann Gesuelli, William J Brunken
The retina expresses several laminins in the outer plexiform layer (OPL), where they may provide an extracellular scaffold for synapse stabilization. Mice with a targeted deletion of the laminin β2 gene (Lamb2) exhibit retinal disruptions: photoreceptor synapses in the OPL are disorganized and the retinal physiological response is attenuated. We hypothesize that laminins are required for proper trans-synaptic alignment. To test this, we compared the distribution, expression, association and modification of several pre- and post-synaptic elements in wild-type and Lamb2-null retinae...
October 12, 2017: Journal of Comparative Neurology
https://www.readbyqxmd.com/read/29018955/the-effect-of-laminin-1-on-enteric-neural-crest-derived-cell-migration-in-the-hirschsprung-s-disease-mouse-model
#10
Nana Nakazawa-Tanaka, N Fujiwara, K Miyahara, S Nakada, E Arikawa-Hirasawa, C Akazawa, M Urao, A Yamataka
BACKGROUND/AIM: Laminin-1 regulates neurite outgrowth in various neuronal cells. We have previously demonstrated that laminin-1 promotes enteric neural crest-derived cell (ENCC) migration by using Sox10-VENUS transgenic mice, in which ENCCs are labeled with a green fluorescent protein, Venus. Mice lacking the endothelin-B receptor gene, Ednrb (-/-) mice, are widely used as a model for Hirschsprung's disease (HD). The aim of this study was to investigate the effects of laminin-1on ENCC migration in Sox10-VENUS(+)/Ednrb (-/-) mice, a newly created HD mice model...
October 10, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/29017538/hgf-potentiates-extracellular-matrix-driven-migration-of-human-myoblasts-involvement-of-matrix-metalloproteinases-and-mapk-erk-pathway
#11
Mariela Natacha González, Wallace de Mello, Gillian S Butler-Browne, Suse Dayse Silva-Barbosa, Vincent Mouly, Wilson Savino, Ingo Riederer
BACKGROUND: The hepatocyte growth factor (HGF) is required for the activation of muscle progenitor cells called satellite cells (SC), plays a role in the migration of proliferating SC (myoblasts), and is present as a soluble factor during muscle regeneration, along with extracellular matrix (ECM) molecules. In this study, we aimed at determining whether HGF is able to interact with ECM proteins, particularly laminin 111 and fibronectin, and to modulate human myoblast migration. METHODS: We evaluated the expression of the HGF-receptor c-Met, laminin, and fibronectin receptors by immunoblotting, flow cytometry, or immunofluorescence and used Transwell assays to analyze myoblast migration on laminin 111 and fibronectin in the absence or presence of HGF...
October 10, 2017: Skeletal Muscle
https://www.readbyqxmd.com/read/28992774/treatment-of-a-burn-animal-model-with-functionalized-tridimensional-electrospun-biomaterials
#12
Daniela Steffens, Monica Beatriz Mathor, Paula Rigon da Luz Soster, Gustavo Vergani, Dayane Piffer Luco, Patricia Pranke
Laminin-functionalized poly-d,l-lactic acid scaffolds were produced. Following this, mesenchymal stem cells and keratinocytes were seeded on biomaterials for the in vivo experiments, where the biomaterials with or without cells were implanted. The analysis is comprised of the visual aspect and mean size of the lesion plus the histology and gene expression. The results showed that the cells occupied all the structure of the scaffolds in all the groups. After nine days of in vivo experiments, the defect size did not show statistical difference among the groups, although the groups with the poly-d,l-lactic acid/Lam biomaterial had the lowest lesion size and presented the best visual aspect of the wound...
January 1, 2017: Journal of Biomaterials Applications
https://www.readbyqxmd.com/read/28992287/new-insights-into-the-evolution-of-the-w-chromosome-in-lepidoptera
#13
Martina Dalíková, Magda Zrzavá, Irena Hladová, Petr Nguyen, Ivan Šonský, Martina Flegrová, Svatava Kubícková, Anna Voleníková, Akito Y Kawahara, Ralph S Peters, František Marec
Moths and butterflies (Lepidoptera) represent the most diverse group of animals with heterogametic females. Although the vast majority of species has a WZ/ZZ (female/male) sex chromosome system, it is generally accepted that the ancestral system was Z/ZZ and the W chromosome has evolved in a common ancestor of Tischeriidae and Ditrysia. However, the lack of data on sex chromosomes in lower Lepidoptera has prevented a formal test of this hypothesis. Here, we performed a detailed analysis of sex chromosomes in Tischeria ekebladella (Tischeriidae) and 3 species representing lower Ditrysia, Cameraria ohridella (Gracillariidae), Plutella xylostella (Plutellidae), and Tineola bisselliella (Tineidae)...
July 18, 2017: Journal of Heredity
https://www.readbyqxmd.com/read/28988991/bifunctional-hydrogels-containing-the-laminin-motif-ikvav-promote%C3%A2-neurogenesis
#14
Aleeza Farrukh, Felipe Ortega, Wenqiang Fan, Nicolás Marichal, Julieta I Paez, Benedikt Berninger, Aránzazu Del Campo, Marcelo J Salierno
Engineering of biomaterials with specific biological properties has gained momentum as a means to control stem cell behavior. Here, we address the effect of bifunctionalized hydrogels comprising polylysine (PL) and a 19-mer peptide containing the laminin motif IKVAV (IKVAV) on embryonic and adult neuronal progenitor cells under different stiffness regimes. Neuronal differentiation of embryonic and adult neural progenitors was accelerated by adjusting the gel stiffness to 2 kPa and 20 kPa, respectively. While gels containing IKVAV or PL alone failed to support long-term cell adhesion, in bifunctional gels, IKVAV synergized with PL to promote differentiation and formation of focal adhesions containing β1-integrin in embryonic cortical neurons...
September 28, 2017: Stem Cell Reports
https://www.readbyqxmd.com/read/28983681/altered-expression-of-laminin-alpha1-in-aganglionic-colon-of-endothelin-receptor-b-null-mouse-model-of-hirschsprung-s-disease
#15
Naho Fujiwara, Nana Nakazawa-Tanaka, Katsumi Miyahara, Eri Arikawa-Hirasawa, Chihiro Akazawa, Atsuyuki Yamataka
PURPOSE: Laminin, an extracellular matrix molecule, is essential for normal development of the nervous system. The alpha1 subunit of laminin-1 (LAMA1) has been reported to promote neurites and outgrowth and is expressed only during embryogenesis. Previously, we developed a Sox10 transgenic version of the Endothelin receptor-B (Ednrb) mouse to visualize Enteric neural crest-derived cell (ENCC)s with a green fluorescent protein, Venus. We designed this study to investigate the expression of LAMA1 using Sox10-VENUS mice gut...
October 5, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28981898/overexpression-of-truncated-human-disc1-induces-appearance-of-hindbrain-oligodendroglia-in-the-forebrain-during-development
#16
Pavel Katsel, Peter Fam, Weilun Tan, Sonia Khan, Chunxia Yang, Yan Jouroukhin, Sergei Rudchenko, Mikhail V Pletnikov, Vahram Haroutunian
Genetic, neuroimaging, and gene expression studies suggest a role for oligodendrocyte (OLG) dysfunction in schizophrenia (SZ). Disrupted-in-schizophrenia 1 (DISC1) is a risk gene for major psychiatric disorders, including SZ. Overexpression of mutant truncated (hDISC1), but not full-length sequence of human DISC1 in forebrain influenced OLG differentiation and proliferation of glial progenitors in the developing cerebral cortex concurrently with reduction of OLG progenitor markers in the hindbrain. We examined gene and protein expression of the molecular determinants of hindbrain OLG development and their interactions with DISC1 in mutant hDISC1 mice...
August 18, 2017: Schizophrenia Bulletin
https://www.readbyqxmd.com/read/28981121/synthesis-and-characterization-of-well-defined-hydrogel-matrices-and-their-application-to-intestinal-stem-cell-and-organoid-culture
#17
Nikolce Gjorevski, Matthias P Lutolf
Growing cells within an extracellular matrix-like 3D gel is required for, or can improve, the growth of many cell types ex vivo. Here, we describe a protocol for the generation of well-defined matrices for the culture of intestinal stem cells (ISCs) and intestinal organoids. These matrices comprise a poly(ethylene glycol) (PEG) hydrogel backbone functionalized with minimal adhesion cues including RGD (Arg-Gly-Asp), which is sufficient for ISC expansion, and laminin-111, which is required for organoid formation...
November 2017: Nature Protocols
https://www.readbyqxmd.com/read/28980742/nintedanib-treatment-delays-prostate-dorsolateral-lobe-cancer-progression-in-the-tramp-model-contribution-to-the-epithelial-stromal-interaction-balance
#18
Ellen Nogueira Pangrazi, Raquel Frenedoso da Silva, Larissa Akemi Kido, Fabio Montico, Valéria Helena Alves Cagnon
Prostate cancer progression mechanism has been linked to epithelial proliferation, tumor invasion ability and growth factors. Nintedanib (BIBF 1120) has been reported as being FGF and VEGF pathway inhibitors, exhibiting antitumor activity. Thus, the objective herein was to characterize the early Nintedanib treatment effects on the structure and molecules involved in the basal membrane, the extracellular matrix maintenance, in addition to the angiogenesis and mitogenic processes at different grades of prostatic tumor development in TRAMP mice...
October 5, 2017: Cell Biology International
https://www.readbyqxmd.com/read/28977852/loss-of-calponin-h1-confers-anoikis-resistance-and-tumor-progression-in-the-development-of-high-grade-serous-carcinoma-originating-from-the-fallopian-tube-epithelium
#19
Kai-Hung Wang, Sung-Chao Chu, Tang-Yuan Chu
Increasing evidence indicates that ovarian high-grade serous carcinoma (HGSC) originates from the fallopian tube epithelium and metastasizes to the ovary as the secondary site. A working hypothesis is that detached tubal HGSC cells survive anoikis and implant on the ovary. In this study, we found that downregulation of calponin h1 (CNN1) is necessary for the anoikis survival and cell transformation. CNN1 was progressively downregulated in cells and tissues representing different stages of HGSC development from fallopian tube epithelium (FTE)...
September 22, 2017: Oncotarget
https://www.readbyqxmd.com/read/28975768/a-physicochemically-optimized-and-neuroconductive-biphasic-nerve-guidance-conduit-for-peripheral-nerve-repair
#20
Alan J Ryan, William A Lackington, Alan J Hibbitts, Austyn Matheson, Tijna Alekseeva, Anna Stejskalova, Phoebe Roche, Fergal J O'Brien
Clinically available hollow nerve guidance conduits (NGCs) have had limited success in treating large peripheral nerve injuries. This study aims to develop a biphasic NGC combining a physicochemically optimized collagen outer conduit to bridge the transected nerve, and a neuroconductive hyaluronic acid-based luminal filler to support regeneration. The outer conduit is mechanically optimized by manipulating crosslinking and collagen density, allowing the engineering of a high wall permeability to mitigate the risk of neuroma formation, while also maintaining physiologically relevant stiffness and enzymatic degradation tuned to coincide with regeneration rates...
October 4, 2017: Advanced Healthcare Materials
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