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https://www.readbyqxmd.com/read/28933358/substrate-deprivation-therapy-to-reduce-glycosaminoglycan-synthesis-improves-aspects-of-neurological-and-skeletal-pathology-in-mps-i-mice
#1
Ainslie L K Derrick-Roberts, Matilda R Jackson, Carmen E Pyragius, Sharon Byers
Mucopolysaccharidosis type I (MPS I) is the most common form of the MPS group of genetic diseases. MPS I results from a deficiency in the lysosomal enzyme α-l-iduronidase, leading to accumulation of undegraded heparan and dermatan sulphate glycosaminoglycan (GAG) chains in patient cells. MPS children suffer from multiple organ failure and die in their teens to early twenties. In particular, MPS I children also suffer from profound mental retardation and skeletal disease that restricts growth and movement. Neither brain nor skeletal disease is adequately treated by current therapy approaches...
February 23, 2017: Diseases (Basel)
https://www.readbyqxmd.com/read/28933195/biophysical-stimuli-a-review-of-electrical-and-mechanical-stimulation-in-hyaline-cartilage
#2
Juan J Vaca-González, Johana M Guevara, Miguel A Moncayo, Hector Castro-Abril, Yoshie Hata, Diego A Garzón-Alvarado
Objective Hyaline cartilage degenerative pathologies induce morphologic and biomechanical changes resulting in cartilage tissue damage. In pursuit of therapeutic options, electrical and mechanical stimulation have been proposed for improving tissue engineering approaches for cartilage repair. The purpose of this review was to highlight the effect of electrical stimulation and mechanical stimuli in chondrocyte behavior. Design Different information sources and the MEDLINE database were systematically revised to summarize the different contributions for the past 40 years...
September 1, 2017: Cartilage
https://www.readbyqxmd.com/read/28930185/bioprospecting-for-exopolysaccharides-from-deep-sea-hydrothermal-vent-bacteria-relationship-between-bacterial-diversity-and-chemical-diversity
#3
Christine Delbarre-Ladrat, Marcia Leyva Salas, Corinne Sinquin, Agata Zykwinska, Sylvia Colliec-Jouault
Many bacteria biosynthesize structurally diverse exopolysaccharides (EPS) and excrete them into their surrounding environment. The EPS functional features have found many applications in industries such as cosmetics and pharmaceutics. In particular, some EPS produced by marine bacteria are composed of uronic acids, neutral sugars, and N-acetylhexosamines, and may also bear some functional sulfate groups. This suggests that they can share common structural features with glycosaminoglycans (GAG) like the two EPS (HE800 and GY785) originating from the deep sea...
September 20, 2017: Microorganisms
https://www.readbyqxmd.com/read/28926869/purification-and-characterization-of-the-soluble-form-of-the-receptor-for-advanced-glycation-end-products-srage-a-novel-fast-economical-and-convenient-method
#4
Varun Kumar, Alba Sulaj, Thomas Fleming, Peter P Nawroth
The receptor for advanced glycation end-products (RAGE) is a multi-ligand receptor which belongs to the pattern recognition receptor family and can bind to various ligands such as advanced glycation end-products (AGEs), members of the S100 protein family, glycosaminoglycans, amyloid β peptides, high-mobility group box-1 (HMGB1) and nucleic acids through its extracellular domain. The RAGE-ligand interaction leads to the activation of MAP kinase and NF-kB signaling pathways. Further ligand-induced up-regulation of RAGE is involved in various patho-physiological situations including late diabetic complications, Alzheimer disease and several other neurodegenerative diseases...
September 19, 2017: Experimental and Clinical Endocrinology & Diabetes
https://www.readbyqxmd.com/read/28925907/atrial-fibrillation-ablation-increases-inflammation-chemokine-modulation-suppresses-activation-of-leukocytes-isolated-after-ablation
#5
Alexandra Lucas, Mohammad Al-Ani, Sriram Ambadapadi, Jordan R Yaron, Donghang Zheng, Adisson Fortunel, Maia Doroton-Guevara, Liying Liu, Sufi Morshed, Jay Fricker, William Miles, Matthew McKillop
AIMS: Atrial fibrillation (AF) ablation is associated with increased circulating markers of inflammation. Innate immune, or inflammation, pathways up-regulate mononuclear cell responses and may increase risk for recurrent arrhythmia. Chemokines and serine protease coagulation pathways both activate innate immune responses. Here, we measured inflammatory markers in peripheral blood samples from patients after cryoballoon and/or radiofrequency pulmonary vein isolation and assessed the capacity for inhibition of chemokine and serine protease pathways to block cell activation...
September 18, 2017: Cardiovascular & Hematological Disorders Drug Targets
https://www.readbyqxmd.com/read/28925497/the-feasibility-of-the-cd271-and-cd271-mesenchymal-stromal-cell-enrichment-toward-nucleus-pulposus-like-cells
#6
Katarzyna Jezierska-Wozniak, Monika Barczewska, Aleksandra Habich, Pawel Wojtacha, Wanda Badowska, Wojciech Maksymowicz, Joanna Wojtkiewicz
INTRODUCTION: Factors promoting nerve cell ingrowth are considered responsible for chronic back pain resulting from the intervertebral disc degeneration (IDD). One of the recent exploratory IDD treatments is stem cell transplantation therapy. The CD271 (low-affinity nerve growth factor receptor) has been identified as a marker of the most homogeneous mesenchymal stem cell (MSC) subset. It is capable of promoting differentiation along adipogenic, osteogenic and chondrogenic lineages and producing significantly higher levels of cytokines as compared to the total population of plastic adherence-mesenchymal stem cells (PA-MSCs)...
September 19, 2017: Folia Histochemica et Cytobiologica
https://www.readbyqxmd.com/read/28922722/molecular-mechanisms-of-anticancer-effects-of-glucosamine
#7
REVIEW
Fatemeh Zahedipour, Razieh Dalirfardouei, Gholamreza Karimi, Khadijeh Jamialahmadi
Glucosamine is an amino sugar that is produced naturally in human body. It is an essential carbohydrate component of many cellular glycoproteins, glycolipids, and glycosaminoglycans (GAGs). This popular over-the-counter supplement is also found in the exoskeleton of crustaceans. Glucosamine and its derivatives have a long history in medicine for inflammatory conditions specially to relieve arthritis. This dietary supplement has numerous biological and pharmacological properties, including anti-inflammatory, antioxidant, anti-aging, anti-fibrotic, neuroprotective and cardioprotective activities...
September 13, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28921412/how-close-are-we-to-therapies-for-sanfilippo-disease
#8
REVIEW
Lidia Gaffke, Karolina Pierzynowska, Ewa Piotrowska, Grzegorz Węgrzyn
Sanfilippo disease is one of mucopolysaccharidoses (MPS), a group of lysosomal storage diseases characterized by accumulation of partially degraded glycosaminoglycans (GAGs). It is classified as MPS type III, though it is caused by four different genetic defects, determining subtypes A, B, C and D. In each subtype of MPS III, the primary storage GAG is heparan sulfate (HS), but mutations leading to A, B, C, and D subtypes are located in genes coding for heparan N-sulfatase (the SGSH gene), α-N-acetylglucosaminidase (the NAGLU gene), acetyl-CoA:α-glucosaminide acetyltransferase (the HGSNAT gene), and N-acetylglucosamine-6-sulfatase (the GNS gene), respectively...
September 18, 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/28920945/neutrophils-a-cornerstone-of-liver-ischemia-and-reperfusion-injury
#9
Thiago Henrique Caldeira de Oliveira, Pedro Elias Marques, Paul Proost, Mauro Martins M Teixeira
Ischemia-reperfusion injury (IRI) is the main cause of morbidity and mortality due to graft rejection after liver transplantation. During IRI, an intense inflammatory process occurs in the liver. This hepatic inflammation is initiated by the ischemic period but occurs mainly during the reperfusion phase, and is characterized by a large neutrophil recruitment to the liver. Production of cytokines, chemokines, and danger signals results in activation of resident hepatocytes, leukocytes, and Kupffer cells. The role of neutrophils as the main amplifiers of liver injury in IRI has been recognized in many publications...
September 18, 2017: Laboratory Investigation; a Journal of Technical Methods and Pathology
https://www.readbyqxmd.com/read/28919746/in-vitro-and-in-vivo-studies-of-a-novel-bacterial-cellulose-based-acellular-bilayer-nanocomposite-scaffold-for-the-repair-of-osteochondral-defects
#10
Jyoti V Kumbhar, Sachin H Jadhav, Dhananjay S Bodas, Amruta Barhanpurkar-Naik, Mohan R Wani, Kishore M Paknikar, Jyutika M Rajwade
Bacterial cellulose (BC) is a naturally occurring nanofibrous biomaterial which exhibits unique physical properties and is amenable to chemical modifications. To explore whether this versatile material can be used in the treatment of osteochondral defects (OCD), we developed and characterized novel BC-based nanocomposite scaffolds, for example, BC-hydroxyapatite (BC-HA) and BC-glycosaminoglycans (BC-GAG) that mimic bone and cartilage, respectively. In vitro biocompatibility of BC-HA and BC-GAG scaffolds was established using osteosarcoma cells, human articular chondrocytes, and human adipose-derived mesenchymal stem cells...
2017: International Journal of Nanomedicine
https://www.readbyqxmd.com/read/28918752/correlation-of-csf-flow-using-phase-contrast-mri-with-ventriculomegaly-and-csf-opening-pressure-in-mucopolysaccharidoses
#11
Amauri Dalla Corte, Carolina F M de Souza, Maurício Anés, Fabio K Maeda, Armelle Lokossou, Leonardo M Vedolin, Maria Gabriela Longo, Monica M Ferreira, Solanger G P Perrone, Olivier Balédent, Roberto Giugliani
BACKGROUND: Very little is known about the incidence and prevalence of hydrocephalus in patients with mucopolysaccharidoses (MPS). The biggest challenge is to distinguish communicating hydrocephalus from ventricular dilatation secondary to brain atrophy, because both conditions share common clinical and neuroradiological features. The main purpose of this study is to assess the relationship between ventriculomegaly, brain and cerebrospinal fluid (CSF) volumes, aqueductal and cervical CSF flows, and CSF opening pressure in MPS patients, and to provide potential biomarkers for abnormal CSF circulation...
September 18, 2017: Fluids and Barriers of the CNS
https://www.readbyqxmd.com/read/28917941/skin-regeneration-effect-of-the-glycosaminoglycans-from-liparis-tessellatus-eggs
#12
Jae-Joon Lee, Bernadeth F Ticar, Zuliyati Rohmah, Si-Hyang Park, Dawon Kang, Seok-Joong Kang, Byeong-Dae Choi
A purified Glycosaminoglycans (GAGs) extract from L. tessellatus eggs was enzyme-hydrolyzed and then fractionated with DEAE-Sepharose column chromatography. The fractions were subsequently subjected to skin regeneration effects analysis against skin fibroblast (CCD-986sk) cell lines. Fraction 3 is evidently to have tyrosinase inhibition activity by 20.1% at concentration of 50mg/mL. DOPA oxidation assay, collagenase inhibition activity assay, fibroblast proliferation assay, and production of type I C-peptide assays were done to further proof the skin regeneration effect of GAGs fractions...
September 13, 2017: International Journal of Biological Macromolecules
https://www.readbyqxmd.com/read/28917911/injectable-glycosaminoglycan-protein-nano-complex-in-semi-interpenetrating-networks-a-biphasic-hydrogel-for-hyaline-cartilage-regeneration
#13
Janani Radhakrishnan, Anuradha Subramanian, Swaminathan Sethuraman
Articular hyaline cartilage regeneration remains challenging due to its less intrinsic reparability. The study develops injectable biphasic semi-interpenetrating polymer networks (SIPN) hydrogel impregnated with chondroitin sulfate (ChS) nanoparticles for functional cartilage restoration. ChS loaded zein nanoparticles (∼150nm) prepared by polyelectrolyte-protein complexation were interspersed into injectable SIPNs developed by blending alginate with poly(vinyl alcohol) and calcium crosslinking. The hydrogel exhibited interconnected porous microstructure (39...
November 1, 2017: Carbohydrate Polymers
https://www.readbyqxmd.com/read/28917905/characterization-of-a-water-soluble-chitosan-derivative-and-its-potential-for-submucosal-injection-in-endoscopic-techniques
#14
Hidemi Hattori, Hironori Tsujimoto, Kazuo Hase, Masayuki Ishihara
To examine the potential of chitosan-based agents for submucosal injection in endoscopic techniques, a chitosan derivative was prepared with lactose moieties linked to the amino groups of its glucosamine units (CH-LA). After dissolving CH-LA in neutral pH solutions, including physiological saline (CH-LA-S), its response to different concentrations of anionic glycosaminoglycans and proteins in the surrounding environment was examined. The CH-LA-S form changed in the presence of sulfated glycosaminoglycans (heparin, chondroitin sulfate, and mucin) and protein (fibrinogen)...
November 1, 2017: Carbohydrate Polymers
https://www.readbyqxmd.com/read/28915283/clinical-evaluation-of-a-multi-modal-facial-serum-that-addresses-hyaluronic-acid-levels-in-skin
#15
Susana Raab, Margarita Yatskayer, Stephen Lynch, Megan Manco, Christian Oresajo
BACKGROUND: Hyaluronic acid (HA), the major glycosaminoglycan present in the human skin, is a key contributor to water retention and mechanical support in skin. The level, size, and functionality of cutaneous HA are known to diminish with age. Topical treatments designed to increase the HA content of skin have been met with limited success. The purpose of this study was to evaluate the tolerance and efficacy of a multi-modal facial serum containing HA, Proxylane (C-Xyloside), purple rice extract, and dipotassium glycyrrhizate in addressing HA levels in skin...
September 1, 2017: Journal of Drugs in Dermatology: JDD
https://www.readbyqxmd.com/read/28914997/synergistic-effect-of-l-ascorbic-acid-and-hyaluronic-acid-on-the-expressions-of-matrix-metalloproteinase-3-and-9-in-human-chondrocytes
#16
Teng-Le Huang, Che-Hua Yang, Goichi Yanai, Jo-Yu Liao, Shoichiro Sumi, Kai-Chiang Yang
Proinflammatory cytokines and reactive oxygen species (ROS) are known to be involved in the initiation and progression of osteoarthritis (OA). New evidence clarifying the correlation between ROS and inflammation has indicated that oxidative stress can up-regulate inflammatory cytokines. l-Ascorbic acid (AA), an antioxidant, has been shown to have anti-inflammatory effects and improve matrix deposition in chondrocytes. The purpose of this study was to examine the effects of hyaluronic acid (HA; 100 μg/mL) supplemented with AA (50 μg/mL) on human normal and interleukin-1 beta-stimulated (IL-1β, 10 ng/mL) chondrocytes...
September 15, 2017: Journal of Biomedical Materials Research. Part B, Applied Biomaterials
https://www.readbyqxmd.com/read/28914427/the-clinical-and-genetic-spectrum-of-maroteaux-lamy-syndrome-mucopolysaccharidosis-vi-in-the-eastern-province-of-saudi-arabia
#17
Nouriya Abbas Al-Sannaa, Hind Yousif Al-Abdulwahed, Sami Ibrahim Al-Majed, Issam Hassan Bouholaigah
Mucopolysaccharidosis (MPS VI) or Maroteaux-Lamy syndrome is an autosomal recessive lysosomal storage disease caused by deficiency of the enzyme N-acetylgalactosamine 4-sulfatase or arylsulfatase B. It is involved in the degradation of glycosaminoglycans and characterized by a wide spectrum of clinical and genetic heterogeneity. So far, more than 150 mutations have been reported in the ARSB gene. Most of these mutations are either novel, private, or compound heterozygous making phenotype-genotype correlation as well as population screening difficult...
September 15, 2017: Journal of Community Genetics
https://www.readbyqxmd.com/read/28913873/improvement-of-decellularization-efficiency-of-porcine-aorta-using-dimethyl-sulfoxide-as-a-penetration-enhancer
#18
Selcan Guler, Halil M Aydin, Lan-Xin Lü, Ying Yang
Decellularization of tissues and organs enables researchers to obtain extracellular matrix (ECM) with the natural conformation and chemical composition of specific tissues. However, drawbacks exist such as the structural alteration of ECM or loss of some important components in ECM due to overexposure to chemicals during the decellularization process. In this study, porcine aorta was decellularized by sodium dodecyl sulfate (SDS). Dimethyl sulfoxide (DMSO) was used as a penetration enhancer in the decellularization process to enhance the penetration of SDS, consequently reducing the exposure time of SDS to treated tissues...
September 15, 2017: Artificial Organs
https://www.readbyqxmd.com/read/28905328/lessons-learned-from-protein-aggregation-toward-technological-and-biomedical-applications
#19
REVIEW
César L Avila, Silvina Chaves, Sergio B Socias, Esteban Vera-Pingitore, Florencia González-Lizárraga, Cecilia Vera, Diego Ploper, Rosana Chehín
The close relationship between protein aggregation and neurodegenerative diseases has been the driving force behind the renewed interest in a field where biophysics, neurobiology and nanotechnology converge in the study of the aggregate state. On one hand, knowledge of the molecular principles that govern the processes of protein aggregation has a direct impact on the design of new drugs for high-incidence pathologies that currently can only be treated palliatively. On the other hand, exploiting the benefits of protein aggregation in the design of new nanomaterials could have a strong impact on biotechnology...
September 13, 2017: Biophysical Reviews
https://www.readbyqxmd.com/read/28904997/development-of-inflammatory-bowel-disease-is-linked-to-a-longitudinal-restructuring-of-the-gut-metagenome-in-mice
#20
Thomas Sharpton, Svetlana Lyalina, Julie Luong, Joey Pham, Emily M Deal, Courtney Armour, Christopher Gaulke, Shomyseh Sanjabi, Katherine S Pollard
The gut microbiome is linked to inflammatory bowel disease (IBD) severity and altered in late-stage disease. However, it is unclear how gut microbial communities change over the course of IBD development, especially in regard to function. To investigate microbiome-mediated disease mechanisms and discover early biomarkers of IBD, we conducted a longitudinal metagenomic investigation in an established mouse model of IBD, where damped transforming growth factor β (TGF-β) signaling in T cells leads to peripheral immune activation, weight loss, and severe colitis...
September 2017: MSystems
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