scholarly journals A Guided Tour of the Structural Biology of Gaucher Disease: Acid-β-Glucosidase and Saposin C

2011 ◽  
Vol 2011 ◽  
pp. 1-15 ◽  
Author(s):  
Raquel L. Lieberman
Keyword(s):  
Crystal Structures ◽  
Structural Biology ◽  
Gaucher Disease ◽  
Lysosomal Storage ◽  
The Past ◽  
Current State ◽  
Saposin C ◽  
Prevalent Disease ◽  
Guided Tour ◽  
Insight Into

Mutations in both acid-β-glucosidase (GCase) and saposin C lead to Gaucher disease, the most common lysosomal storage disorder. The past several years have seen an explosion of structural and biochemical information for these proteins, which have provided new insight into the biology and pathogenesis of Gaucher disease, as well as opportunities for new therapeutic directions. Nearly 20 crystal structures of GCase are now available, from different heterologous sources, complexed with different ligands in the active site, in different glycosylation states, as well as one that harbors a prevalent disease-causing mutation, N370S. For saposin C, two NMR and 3 crystal structures have been solved, each with its unique snapshot. This review focuses on the details of these structures to highlight salient common and disparate features that contribute to our current state of knowledge of this complex orphan disease.

¿¿Nueva narrativa? Entrevista con David Toscana

2002 ◽  
Vol 18 (2) ◽  
pp. 351-362
Author(s):  
Pablo A. J. Brescia ◽  
Scott M. Bennett
Keyword(s):  
Latin American ◽  
The Past ◽  
Current State ◽  
Latin American Narrative ◽  
Boom Latinoamericano ◽  
The 1960S ◽  
Time Frames ◽  
Insight Into

This interview with Mexican writer David Toscana ponders the current state of both Mexican and Latin American narrative and serves as an insight into his own works. Toscana's statements about the recurring themes in his narrative (failure, loneliness, characters put to the test, and a tendency to play with different time frames, especially in his novels) help illustrate some of the characteristics of his fiction, which, according to various critics, is one of the most promising today in Mexico. Also noteworthy are his comments about the tendency of writers from his generation (born in the 1960s and later) to reject the legacy of the "boom" writers. Toscana's own interest is to revisit history and tradition by constructing a different voice and a different vision, a new way of seeing and hearing both the past and the present. Esta entrevista con el escritor mexicano David Toscana trata de explorar el estado actual de la narrativa mexicana y latinoamericana, améén de servir como una aproximacióón a la incipiente obra de este autor. Las respuestas de Toscana sobre los temas recurrentes en su literatura (el fracaso, la soledad, los personajes sometidos a pruebas y una tendencia a proponer diferentes marcos temporales, especialmente en sus novelas) subrayan algunos rasgos de su ficcióón, la cual, segúún la críítica especializada, es una de las máás prometedoras en el panorama mexicano de hoy. De especial interéés son los comentarios de Toscana sobre la tendencia de los escritores de su generacióón a rechazar el legado del "boom" latinoamericano. Toscana reacciona contra ese rechazo y propone una voz personal que vuelva a la historia y a la tradicióón y plantee una nueva manera de ver el pasado y el presente de Mééxico.

New developments in the theory and interpretation of X-ray spectra based on fast parallel calculations

2002 ◽  
Vol 10 (1) ◽  
pp. 43-45 ◽  
Author(s):  
J. J. Rehr ◽  
A. L. Ankudinov
Keyword(s):  
Ab Initio ◽  
Structural Biology ◽  
Significant Progress ◽  
Edge Structure ◽  
New Developments ◽  
X Ray ◽  
The Past ◽  
Current State ◽  
X Ray Absorption ◽  
Made In

There has been dramatic progress over the past decade both in theory and inab initiocalculations of X-ray absorption fine structure. Significant progress has also been made in understanding X-ray absorption near-edge structure (XANES). This contribution briefly reviews the developments in this field leading up to the current state. One of the key advances has been the development of severalab initiocodes such asFEFF, which permit an interpretation of the spectra in terms of geometrical and electronic properties of a material. Despite this progress, XANES calculations have remained challenging both to compute and to interpret. However, recent advances based on parallel Lanczos multiple-scattering algorithms have led to speed increases of typically two orders of magnitude, making fast calculations practicable. Improvements in the interpretation of near-edge structure have also been made. It is suggested that these developments can be advantageous in structural biology,e.g.in post-genomics studies of metalloproteins.

scholarly journals Circular RNAs in osteoarthritis: indispensable regulators and novel strategies in clinical implications

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Wenchao Zhang ◽  
Lin Qi ◽  
Ruiqi Chen ◽  
Jieyu He ◽  
Zhongyue Liu ◽  
...  
Keyword(s):  
Hot Spot ◽  
Circular Rnas ◽  
Clinical Implications ◽  
Biological Processes ◽  
Future Directions ◽  
The Past ◽  
Prevalent Disease ◽  
Post Traumatic ◽  
Socioeconomic Burden ◽  
Insight Into

AbstractOver the past decades, circular RNAs (circRNAs) have emerged as a hot spot and sparked intensive interest. Initially considered as the transcriptional noises, further studies have indicated that circRNAs are crucial regulators in multiple cellular biological processes, and thus engage in the development and progression of many diseases including osteoarthritis (OA). OA is a prevalent disease that mainly affects those aging, obese and post-traumatic population, posing as a major source of socioeconomic burden. Recently, numerous circRNAs have been found aberrantly expressed in OA tissues compared with counterparts. More importantly, circRNAs have been demonstrated to interplay with components in OA microenvironments, such as chondrocytes, synoviocytes and macrophages, by regulation of their proliferation, apoptosis, autophagy, inflammation, or extracellular matrix reorganization. Herein, in this review, we extensively summarize the roles of circRNAs in OA microenvironment, progression, and putative treatment, as well as envision the future directions for circRNAs research in OA, with the aim to provide a novel insight into this field.

scholarly journals Two-dimensional imaging detectors for structural biology with X-ray lasers

2014 ◽  
Vol 369 (1647) ◽  
pp. 20130334 ◽  
Author(s):  
Peter Denes
Keyword(s):  
Structural Biology ◽  
Peak Power ◽  
State Of The Art ◽  
High Peak Power ◽  
Free Electron Lasers ◽  
X Ray ◽  
The Past ◽  
Current State ◽  
Pixel Detectors ◽  
Imaging Detectors

Our ability to harness the advances in microelectronics over the past decade(s) for X-ray detection has resulted in significant improvements in the state of the art. Biology with X-ray free-electron lasers present daunting detector challenges: all of the photons arrive at the same time, and individual high peak power pulses must be read out shot-by-shot. Direct X-ray detection in silicon pixel detectors—monolithic or hybrid—are the standard for XFELs today. For structural biology, improvements are needed for today's 10–100 Hz XFELs, and further improvements are required for tomorrow's 10+ kHz XFELs. This article will discuss detector challenges, why they arise and ways to overcome them, along with the current state of the art.

scholarly journals Gaucher disease: achievements and prospects

2021 ◽  
Vol 93 (7) ◽  
pp. 830-836
Author(s):  
Rodion V. Ponomarev ◽  
Elena A. Lukina
Keyword(s):  
Cell Biology ◽  
Gaucher Disease ◽  
Scientific Community ◽  
Clinical Phenotype ◽  
Lysosomal Storage ◽  
Specific Therapy ◽  
Wide Audience ◽  
Effective Work ◽  
The Past ◽  
Progressive Disorder

Gaucher disease (GD) is the most common lysosomal storage disorder, resulting from a deficiency in the activity of a lysosomal enzyme glucocerebrosidase, which is involved in the catabolism of sphingolipids. The phenomenal progress in understanding the pathogenesis and development of specific therapy of this disease over the past 60 years dramatically changed the clinical phenotype of GD, turning a severe progressive disorder into an asymptomatic metabolic defect. The evolution of the understanding of GD associated with fundamental discoveries in the field of cell biology, biochemistry and genetics may be of interest to a wide audience as a model of the effective work of the scientific community in the treatment of rare metabolic pathology.

Ground-penetrating radar for assessing winter roads

Geophysics ◽  
2016 ◽  
Vol 81 (1) ◽  
pp. WA101-WA109 ◽  
Author(s):  
A. Peter Annan ◽  
Nectaria Diamanti ◽  
J. David Redman ◽  
Steven R. Jackson
Keyword(s):  
Ground Penetrating Radar ◽  
Road Safety ◽  
Thickness Variation ◽  
The Past ◽  
Current State ◽  
Winter Road ◽  
Ground Penetrating ◽  
Transportation Routes ◽  
Answering Questions ◽  
Insight Into

Since its inception, ground penetrating radar (GPR) has been a very effective method for examining ice structure. The vast majority of early GPR use was focused on answering questions related to glaciology and ice sheets. More recently, as more and more activity occurs in arctic areas, managing ice structures for a variety of applications has created new uses for GPR. For the past 30–40 years, GPR use for assessing transportation routes over lakes, rivers, and sea ice has been reported, but, only in the last decade has routine application occurred. We have focused on the application of GPR for winter road safety, explained the operational requirements, discussed the current state of practice, and illustrated modern GPR instrumentation. Based on widespread field observations, we have provided information on the ice-layer thickness variation and the variability on GPR ice bottom reflection. Combining extensive GPR and ice auger data provided insight into the variability of electromagnetic wave velocity in ice. Extensive observations showed that velocity varies with ice thickness, and velocities are often considerably lower than observed in “pure” ice.

Recovery boiler sootblowers: History and technological advances

TAPPI Journal ◽  
2015 ◽  
Vol 14 (1) ◽  
pp. 51-60
Author(s):  
HONGHI TRAN ◽  
DANNY TANDRA
Keyword(s):  
Cfd Modeling ◽  
Computing Systems ◽  
The Past ◽  
Technological Advances ◽  
Recovery Boilers ◽  
Computational Fluid Dynamics Cfd ◽  
Recovery Boiler ◽  
Steam Parameters ◽  
Insight Into ◽  
Deposit Removal

Sootblowing technology used in recovery boilers originated from that used in coal-fired boilers. It started with manual cleaning with hand lancing and hand blowing, and evolved slowly into online sootblowing using retractable sootblowers. Since 1991, intensive research and development has focused on sootblowing jet fundamentals and deposit removal in recovery boilers. The results have provided much insight into sootblower jet hydrodynamics, how a sootblower jet interacts with tubes and deposits, and factors influencing its deposit removal efficiency, and have led to two important innovations: fully-expanded sootblower nozzles that are used in virtually all recovery boilers today, and the low pressure sootblowing technology that has been implemented in several new recovery boilers. The availability of powerful computing systems, superfast microprocessors and data acquisition systems, and versatile computational fluid dynamics (CFD) modeling capability in the past two decades has also contributed greatly to the advancement of sootblowing technology. High quality infrared inspection cameras have enabled mills to inspect the deposit buildup conditions in the boiler during operation, and helped identify problems with sootblower lance swinging and superheater platens and boiler bank tube vibrations. As the recovery boiler firing capacity and steam parameters have increased markedly in recent years, sootblowers have become larger and longer, and this can present a challenge in terms of both sootblower design and operation.

Current state and main directions of preservation and strengthening of health of the working population of Russia

2020 ◽  
pp. 527-532 ◽  
Author(s):  
I. V. Bukhtiyarov
Keyword(s):  
Working Conditions ◽  
Russian Federation ◽  
Production Environment ◽  
Working Population ◽  
Industrial Enterprises ◽  
The Past ◽  
Production Factors ◽  
Current State ◽  
The Russian Federation

The article presents the results of the analysis of health, working conditions and prevalence of adverse production factors, the structure of the detected occupational pathology in the working population of the Russian Federation. The article presents Statistical data on the dynamics of the share of workplaces of industrial enterprises that do not meet hygienic standards, occupational morbidity in 2015-2018 for the main groups of adverse factors of the production environment and the labor process. The indicators of occupational morbidity over the past 6 years in the context of the main types of economic activity, individual subjects of the Russian Federation, classes of working conditions, levels of specialized occupational health care. The role of the research Institute of occupational pathology and occupational pathology centers in solving organizational, methodological and practical tasks for the detection, treatment, rehabilitation and prevention of occupational diseases is shown. The basic directions of activity in the field of preservation and strengthening of health of workers, and also safety at a workplace are defined.

Is there a role for scintigraphic imaging of bone manifestations in Gaucher disease?

2008 ◽  
Vol 47 (06) ◽  
pp. 239-247 ◽  
Author(s):  
S. Kohlfürst ◽  
H. J. Gallowitsch ◽  
E. Kresnik ◽  
P. Lind ◽  
A. B. Mehta ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Lysosomal Storage Disease ◽  
Gaucher Disease ◽  
Storage Disease ◽  
Lysosomal Storage ◽  
Scintigraphic Imaging ◽  
Imaging Methods ◽  
X Ray ◽  
Deficient Activity

SummaryGaucher disease is the most prevalent inherited, lysosomal storage disease and is caused by deficient activity of the enzyme β-glucocerebrosidase. Bone and bone marrow alterations are frequent in the most prevalent non-neuronopathic form of Gaucher disease. Imaging of bone manifestations in Gaucher disease is performed by a variety of imaging methods, conventional X-ray and MRI as the most frequently and most important ones. However, different modalities of scintigraphic imaging have also been used. This article gives an overview on scintigraphic imaging with respect to bone manifestations in Gaucher disease discussing the advantages and limitations of scintigraphic imaging in comparison to other imaging methods.

Phytochemical and Pharmacological Review on Two Herbs of Baijiangcao: Patrinia scabiosaefolia Fisch. ex Trev. and Patrinia villosa (Thunb.) Juss

2020 ◽  
Vol 17 (5) ◽  
pp. 496-517
Author(s):  
Yangcheng Liu ◽  
Wei Liu ◽  
Jiaqi Wang ◽  
Yang Liu ◽  
Changlan Chen ◽  
...  
Keyword(s):  
Chemical Constituents ◽  
Volatile Components ◽  
Research Papers ◽  
Pharmacological Activities ◽  
Comprehensive Review ◽  
Scientific Databases ◽  
The Past ◽  
Current State ◽  
Patrinia Scabiosaefolia ◽  
Antiviral Activities

Patrinia scabiosaefolia Fisch. Trev. and Patrinia villosa (Thunb.) Juss, are two species of Patrinia recorded in the Chinese Pharmacopoeia with the same Chinese name “Baijiangcao” and similar therapeutic effect in traditional Chinese medicine. The present article is the first comprehensive review on the chemical composition and pharmacological activities of these herbs. In this review, data on chemical constituents and pharmacological profile of the two herbs are provided. This review discusses all the classes of the 223 compounds (phenylpropanoids, flavonoids, terpenes, saponins and volatile components, etc.) detected in the two herbs providing information on the current state of knowledge of the phytochemicals present in them. In the past three years, our research group has isolated and identified about more than 100 ingredients from the two herbs. Therefore, we published a systematic review of our research papers and studies on the two herbs were carried out using resources such as classic books about Chinese herbal medicine and scientific databases including Pubmed, Web of Science, SciFinder, CNKI. etc. The present review discusses the most thoroughly studied pharmacological activities (antioxidant, anti-inflammatory, immunomodulatory, antimicrobial, antitumor and antiviral activities) of the two herbs. This comprehensive review will be informative for scientists searching for new properties of these herbs and will be important and significant for the discovery of bioactive compounds from the two herbs and in complete utilization of Patrinia scabiosaefolia Fisch. ex Trev. and Patrinia villosa (Thunb.) Juss.

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