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https://www.readbyqxmd.com/read/29784804/structure-of-xyloglucan-xylosyltransferase-1-reveals-simple-steric-rules-that-define-biological-patterns-of-xyloglucan-polymers
#1
Alan T Culbertson, Jacqueline J Ehrlich, Jun-Yong Choe, Richard B Honzatko, Olga A Zabotina
The plant cell wall is primarily a polysaccharide mesh of the most abundant biopolymers on earth. Although one of the richest sources of biorenewable materials, the biosynthesis of the plant polysaccharides is poorly understood. Structures of many essential plant glycosyltransferases are unknown and suitable substrates are often unavailable for in vitro analysis. The dearth of such information impedes the development of plants better suited for industrial applications. Presented here are structures of Arabidopsis xyloglucan xylosyltransferase 1 (XXT1) without ligands and in complexes with UDP and cellohexaose...
May 21, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29782836/dancing-with-the-diva-hsp90-client-interactions
#2
REVIEW
Martina Radli, Stefan G D Rüdiger
The molecular chaperone Hsp90 is involved in the folding, maturation and degradation of a large number structurally and sequentially unrelated clients, often connected to serious diseases. Elucidating the principles of how Hsp90 recognises this large variety of substrates is essential for comprehending the mechanism of this chaperone machinery, as well as it is a prerequisite for the design of client specific drugs targeting Hsp90. Here, we discuss the recent progress in understanding the substrate recognition principles of Hsp90 and its implications for the role of Hsp90 in the lifecycle of (signalling) molecules...
May 18, 2018: Journal of Molecular Biology
https://www.readbyqxmd.com/read/29782331/management-challenges-in-an-infant-with-pentalogy-of-cantrell-giant-anterior-encephalocele-and-craniofacial-anomalies-a-case-report
#3
Richard Hubbard, Seth Hayes, Holly Gillis, Spencer Lindsey, Prashant Malhotra, Tariq Wani, Joseph D Tobias, Ralph Beltran
Pentalogy of Cantrell is a rare syndrome consisting of midline abnormalities involving the heart, sternum, abdominal wall, and the anterior and pericardial diaphragm. This combination of defects places patients at particular perioperative risk and requires individualized management during anesthetic care. The following report documents the management of a patient with pentalogy of Cantrell, whose condition was further complicated by severe midline craniofacial abnormalities, including large anterior encephalocele, deficient mandible, tethered tongue, and cleft palate...
May 17, 2018: A&A practice
https://www.readbyqxmd.com/read/29781722/survey-of-north-american-multidisciplinary-cleft-palate-and-craniofacial-team-clinic-administration
#4
Kristin D Pfeifauf, Kamlesh B Patel, Alison Snyder-Warwick, Gary B Skolnick, Sibyl Scheve, Sybill D Naidoo
OBJECTIVE: This study aims to provide an understanding of the ways cleft palate (CP) and craniofacial teams address billing, administration, communication of clinical recommendations, appointment scheduling, and diagnosis-specific protocols. DESIGN: An online clinic administration survey was developed using data from an open-ended telephone questionnaire. The online survey was distributed by e-mail to the American Cleft Palate-Craniofacial Association (ACPA) nurse coordinator electronic mailing list, used regularly by the ACPA and its members to communicate with teams...
January 1, 2018: Cleft Palate-craniofacial Journal
https://www.readbyqxmd.com/read/29781512/natural-history-of-rathke-s-cleft-cysts-a-retrospective-analysis-of-a-two-centers-experience
#5
Elisa Sala, Justin M Moore, Alvaro Amorin, Giulia Carosi, Hector Martinez, Griffith R Harsh, Maura Arosio, Giovanna Mantovani, Laurence Katznelson
OBJECTIVE: Rathke's Cleft Cyst (RCC) is a common sellar lesion which may cause visual impairment, hypopituitarism, and headaches from mass effect. The natural history of these lesions is currently unclear. We investigated the natural history of RCCs and compared surgically treated patients with those treated conservatively. METHODS: We performed a retrospective cohort study of patients diagnosed with a RCC between 1996 and 2016 at Stanford University and Ospedale Maggiore Policlinico di Milano...
May 21, 2018: Clinical Endocrinology
https://www.readbyqxmd.com/read/29781248/the-role-of-3d-printing-in-treating-craniomaxillofacial-congenital-anomalies
#6
REVIEW
Christopher D Lopez, Lukasz Witek, Andrea Torroni, Roberto L Flores, David B Demissie, Simon Young, Bruce N Cronstein, Paulo G Coelho
Craniomaxillofacial congenital anomalies comprise approximately one third of all congenital birth defects and include deformities such as alveolar clefts, craniosynostosis, and microtia. Current surgical treatments commonly require the use of autogenous graft material which are difficult to shape, limited in supply, associated with donor site morbidity and cannot grow with a maturing skeleton. Our group has demonstrated that 3D printed bio-ceramic scaffolds can generate vascularized bone within large, critical-sized defects (defects too large to heal spontaneously) of the craniomaxillofacial skeleton...
May 20, 2018: Birth Defects Research
https://www.readbyqxmd.com/read/29780959/a-simple-lens-sparing-technique-to-treat-hypotonic-maculopathy-secondary-to-large-cyclodialysis
#7
Masayo Kimura, Tsutomu Yasukawa, Yuichiro Ogura
Purpose: Hypotonic maculopathy secondary to cyclodialysis often persists and causes irreversible visual loss despite a variety of treatments proposed. The purpose of this study is to report two cases with persistent hypotonic maculopathy due to a large cyclodialysis cleft treated with a simple, lens-sparing technique of external drainage, diathermy, and suturing under the placement of an infusion cannula. Observations: Both patients had sustained blunt trauma to one eye, causing persisting hypotonic maculopathy...
June 2018: American Journal of Ophthalmology Case Reports
https://www.readbyqxmd.com/read/29780930/morpho-functional-evaluation-of-torpedo-maculopathy-with-optical-coherence-tomography-angiography-and-microperimetry
#8
Gabriela Grimaldi, Andrea Scupola, Maria G Sammarco, Michele Marullo, Maria A Blasi
Purpose: To report the case of a 13-year-old girl with torpedo maculopathy, evaluated with multimodal morpho-functional retinal imaging, including fundus photography, infra-red and blue fundus autofluorescence, swept-source optical coherence tomography (OCT), en face OCT, OCT angiography and microperimetry (MP). Observations: On fundus examination, a torpedo-like hypopigmented lesion was observed temporal to the fovea in the left eye. OCT showed disruption of outer retinal layers and the presence of a subretinal cleft...
June 2018: American Journal of Ophthalmology Case Reports
https://www.readbyqxmd.com/read/29780324/intercalated-disk-extracellular-nanodomain-expansion-in-patients-with-atrial-fibrillation
#9
Tristan B Raisch, Matthew S Yanoff, Timothy R Larsen, Mohammed A Farooqui, D Ryan King, Rengasayee Veeraraghavan, Robert G Gourdie, Joseph W Baker, William S Arnold, Soufian T AlMahameed, Steven Poelzing
Aims: Atrial fibrillation (AF) is the most common sustained arrhythmia. Previous evidence in animal models suggests that the gap junction (GJ) adjacent nanodomain - perinexus - is a site capable of independent intercellular communication via ephaptic transmission. Perinexal expansion is associated with slowed conduction and increased ventricular arrhythmias in animal models, but has not been studied in human tissue. The purpose of this study was to characterize the perinexus in humans and determine if perinexal expansion associates with AF...
2018: Frontiers in Physiology
https://www.readbyqxmd.com/read/29780131/bulky-branchial-cleft-cyst-displacing-the-internal-jugular-vein
#10
Ryohei Ono, Izumi Kitagawa, Hiroshi Osafune
No abstract text is available yet for this article.
May 18, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29779274/-outcome-and-influential-factors-of-speech-therapy-delivered-to-cleft-patients-with-post-operative-velopharyngeal-competence
#11
Chun-Li Guo, Heng Yin, Jing-Tao Li, Cheng-Hao Li, Yang Li, Bing Shi, Qian Zheng
OBJECTIVE: To analyze the training technique, intervention timing, and other related factors involved in the speech therapy delivered to cleft patients with velopharyngeal competence after surgery. METHODS: A retrospective study was conducted on 32 patients who received phonology-articulation speech therapies during 2012 to 2013 in Dept. of Cleft Lip and Palate, West China Hospital of Stomatology, Sichuan University. All patients achieved normal speech one year after therapy...
April 1, 2018: Hua Xi Kou Qiang Yi Xue za Zhi, Huaxi Kouqiang Yixue Zazhi, West China Journal of Stomatology
https://www.readbyqxmd.com/read/29778623/hypothesis-driven-versus-hypothesis-free-approaches-to-the-identification-of-genes-for-cleft-lip-and-palate
#12
Alexandre R Vieira
Much progress has been made in understanding the genetic contributions to isolated forms of cleft lip and palate, however this effort is yet to be translated to enhanced genetic counseling or prevention strategies. Interest in the topic was renewed with the ability to perform genome-wide genotyping and after a few of those relatively larger scale studies, many are considering more targeted efforts and to focus on specific genes and/or loci. Here we discuss this crossroad and suggest targeted approaches and rethought clinical descriptions are a better way to move the field forward...
May 16, 2018: Archives of Oral Biology
https://www.readbyqxmd.com/read/29777898/a-survey-of-health-professionals-views-on-acceptable-gestational-age-and-termination-of-pregnancy-for-fetal-anomaly
#13
Lisa Crowe, Ruth H Graham, Stephen C Robson, Judith Rankin
Termination of pregnancy for fetal anomaly is legal in the UK with no upper limit, if two doctors, in good faith, agree "there is a substantial risk that if the child were born it would suffer from such physical or mental abnormalities as to be seriously handicapped". This is Clause E of the Human Fertlisation and Embryology Act. The most commonly sighted Clause is C, which states "the pregnancy has not exceeded its twenty-fourth week and that the continuance of the pregnancy would involve risk, greater than if the pregnancy were terminated, of injury to the physical or mental health of the pregnant woman"...
May 16, 2018: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/29777780/pierre-robin-sequence-a-comprehensive-narrative-review-of-the-literature-over-time
#14
Amerigo Giudice, Selene Barone, Kahina Belhous, Anne Morice, Véronique Soupre, Francesco Bennardo, Nathalie Boddaert, Marie-Paule Vazquez, Véronique Abadie, Arnaud Picard
Pierre Robin syndrome (PRS) is characterized of a triad of clinical signs: micrognathia, glossoptosis and obstruction of the upper airways frequently associated with palatal cleft. It is a heterogenic pathological entity and it can be found as isolated disease (nsPRS) or in association with other syndromes (sPRS), with more pronounced symptoms and systemic involvement. This review aims to summarize the principal features of PRS, analysing the different aspects of the disease. Epidemiological data highlight incidence, severity and mortality of PRS; pathophysiological mechanism reports the etiology and pathogenesis of the disease distinguishing between isolated and syndromic form...
May 16, 2018: Journal of Stomatology, Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/29775557/symptoms-of-obstructive-sleep-apnea-nasal-obstruction-and-enuresis-in-children-with-nonsyndromic-cleft-lip-and-palate-a-prevalence-study
#15
Marilyse de Bragança Lopes Fernandes, Alícia Graziela Noronha Silva Salgueiro, Eliete Janaína Bueno Bighetti, Ivy Kiemle Trindade-Suedam, Inge Elly Kiemle Trindade
OBJECTIVE: To estimate the prevalence of symptoms of obstructive sleep apnea (OSA), nasal obstruction, and enuresis in children with nonsyndromic unilateral cleft lip and palate. DESIGN: Prospective cross-sectional study. SETTING: Referral care center. PARTICIPANTS: One hundred seventy-four children aged 6 to 12 years of both genders. INTERVENTIONS: Symptoms of OSA and nasal obstruction were investigated by analysis of scores obtained by the Sleep Disturbance Scale for Children (SDSC) and Congestion Quantifier (CQ-5)...
January 1, 2018: Cleft Palate-craniofacial Journal
https://www.readbyqxmd.com/read/29775286/multiple-architectures-and-mechanisms-of-latency-in-metallopeptidase-zymogens
#16
Joan L Arolas, Theodoros Goulas, Anna Cuppari, F Xavier Gomis-Rüth
Metallopeptidases cleave polypeptides bound in the active-site cleft of catalytic domains through a general base/acid mechanism. This involves a solvent molecule bound to a catalytic zinc and general regulation of the mechanism through zymogen-based latency. Sixty reported structures from 11 metallopeptidase families reveal that prosegments, mostly N-terminal of the catalytic domain, block the cleft regardless of their size. Prosegments may be peptides (5-14 residues), which are only structured within the zymogens, or large moieties (<227 residues) of one or two folded domains...
May 18, 2018: Chemical Reviews
https://www.readbyqxmd.com/read/29775155/long-term-surgical-outcomes-following-transsphenoidal-surgery-in-patients-with-rathke-s-cleft-cysts
#17
Michelle Lin, Michelle A Wedemeyer, Daniel Bradley, Daniel A Donoho, Vance L Fredrickson, Martin H Weiss, John D Carmichael, Gabriel Zada
OBJECTIVE Rathke's cleft cysts (RCCs) are benign epithelial lesions of the sellar region typically treated via a transsphenoidal approach with cyst fenestration and drainage. At present, there is limited evidence to guide patient selection for operative treatment. Furthermore, there is minimal literature describing factors contributing to cyst recurrence. METHODS The authors conducted a retrospective analysis of 109 consecutive cases of pathology-confirmed RCCs treated via a transsphenoidal approach at a single center from 1995 to 2016...
May 18, 2018: Journal of Neurosurgery
https://www.readbyqxmd.com/read/29774643/a-nona-nuclear-heterometallic-pd3pt6-donut-shaped-cage-molecular-recognition-and-photocatalysis
#18
Dan Preston, Keith C Gordon, Joshua J Sutton, James D Crowley
We report a simple, low symmetry 2-(1-(pyridine-4-methyl)-1H-1,2,3-triazol-4-yl)pyridine ligand which has both monodentate and bidentate binding sites. With platinumII and/or palladiumII ions, two examples of a new nona-nuclear metallo-assembly have been accessed. These complexes have been characterized by nuclear magnetic resonance (NMR) spectroscopies, electrospray mass spectrometry (ESI-MS), and in key cases, X-ray crystallography. The cages possess three clefts comprised of planar cationic panels. This structural feature enables the binding of planar aromatic guests such as anthracene...
May 18, 2018: Angewandte Chemie
https://www.readbyqxmd.com/read/29773124/otolaryngology-and-the-global-burden-of-disease
#19
REVIEW
James E Saunders, Zane Rankin, Kathryn Y Noonan
The Global Burden of Disease (GBD) project provides longitudinal analysis of the global burden of otolaryngologic diseases by measuring the all-cause mortality, years of life lost, the years of life lived with disability, and disability-adjusted life years. Hearing loss burden is assessed overall and as sequelae of other diseases, such as otitis media or meningitis. Using these measures, we can appreciate the high prevalence and disability related to hearing loss globally. Other otolaryngologic diseases that contribute to the GBD include otitis media, cleft lip and palate, head and neck cancer, facial trauma, and oral disorders...
June 2018: Otolaryngologic Clinics of North America
https://www.readbyqxmd.com/read/29772858/-observation-of-clinical-effects-of-percutaneous-vertebroplasty-in-the-treatment-of-vertebral-compression-fractures-complicated-with-intravertebral-clefts
#20
Lei Duan, Guang-Chuan Yang, Wei-Zhong Tang, Jun Zheng
OBJECTIVE: To explore the clinical effects of percutaneous vertebroplasty(PVP)in the treatment of osteoporotic vertebral compression fractures complicated with intravertebral clefts. METHODS: The clinical data of 176 patients with osteoporotic vertebral compression fractures underwent treatment from January 2013 to May 2016 were retrospectively analyzed. All the patients were treated by unilateral PVP procedure, 37 patients with intravertebral clefts. Seven cases were excluded according to the standard and 30 patients were internalized in the study...
April 25, 2018: Zhongguo Gu Shang, China Journal of Orthopaedics and Traumatology
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