fabry dirk smeets and dirk vandermeulen

Báo cáo khoa học: Active-site-speciﬁc chaperone therapy for Fabry disease Yin and Yang of enzyme inhibitors pptx

Ngày tải lên : 30/03/2014, 03:20

... Gaucher disease [20,21], Tay-Sachs and Sandhoff disease [22] (details for Gaucher and Tay-Sachs ⁄ Sandhoff diseases are reviewed separately), GM1-gangliosidosis [23], and retinitis pigmentosa 17 [24] ... contains antiparallel b strands The galactose ligand is shown in yellow and red (B) Fabry disease-causing point mutations are shown on the human a-Gal A dimer The red, blue, and green bonds show mutations ... N215S, Q279E, M296I, and M296V), four mutations found in classic patients (E59K, A156V, L166V, and R356W), and two mutations present in both variant and classic patients (A97V and R301Q) were efﬁciently...

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Organizations social systems conducting experiments 2nd ed jan achterbergh and dirk vriens

Ngày tải lên : 18/11/2016, 11:31

... It appears from the table, that operands A and D have two transforms, B and D, and B and E respectively Operands B, C and E have only one transform, C, A, and A respectively From this description, ... provides concepts for understanding the regulation of any kind of system, and, therefore, it can help to understand and make explicit the regulation of organizations Understanding regulation is important ... regulating and realizing transformations This description of regulating and realizing organizational processes is quite basic and underlies many reflections and studies about organizations and their...

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Tài liệu Báo cáo khoa học: Treatment of neutral glycosphingolipid lysosomal storage diseases via inhibition of the ABC drug transporter, MDR1 Cyclosporin A can lower serum and liver globotriaosyl ceramide levels in the Fabry mouse model doc

Ngày tải lên : 19/02/2014, 07:20

... Wild-type kidney 11 12 13 14 10 Fabry kidney 1 Fabry liver sphingomyelin E D Fabry liver GSLs F 10 G Fig Comparison of Gb3 levels in wild-type and Fabry mouse liver and kidney: relative effect of ... standard; lane 4, GM3 ganglioside standard; (B) lanes 5, 7, 9–11 CsA-treated Fabry mice; lanes 6, 8, 12–14 control Fabry mice; (E) lanes 5, 6, 10, CsA-treated Fabry mice; lanes 7–9, control Fabry ... on tissue Gb3 GSLs were extracted from kidney and liver of control and CsA-treated Fabry mice after weeks recovery post ERT, and from CsA-treated and untreated wildtype mice The Gb3 content was...

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EMBEDDED LINUX SYSTEM DESIGN AND DEVELOPMENT.pdf

Ngày tải lên : 04/08/2012, 14:23

... Ⅲ Understand the embedded Linux development environment Ⅲ Understand and create Linux BSP for a hardware platform Ⅲ Understand the Linux model for embedded storage and write drivers and applications ... memory leaks and memory corruption in applications and drivers Ⅲ Learn methods to proﬁle applications and the kernel Ⅲ Understand uCLinux architecture and its programming model Ⅲ Understand the embedded ... Ltd Ⅲ Windows, WinCE and Microsoft are registered trademarks and MS-DOS and DirectX are trademarks of Microsoft Corporation Ⅲ Solaris and Java are registered trademarks and ChorusOS is a trademark...

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09 Physical and Chemical Characteristics of DDGS revisions.

Ngày tải lên : 08/08/2012, 10:03

... during transport and handling – particle and ingredient segregation (separation) occurs when particles of different sizes and bulk densities are blended together and transported or handled Pellet ... Size, Bulk Density and pH Particle size and particle size uniformity of feed ingredients are important considerations of livestock and poultry nutritionists when selecting sources and determining ... Characteristics of U.S DDGS Physical and chemical characteristics of distiller’s dried grains with solubles (DDGS) vary among sources and can influence its feeding value and handling characteristics These...

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The Art of Public Speaking cylinder, piston, water, and fire, is the steam−engine in its most elementary form. For a steam−engine may be defined as an apparatus for doing work by means of heat applied to water; and since raising such a weight as the pisto

Ngày tải lên : 08/08/2012, 17:20

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CHECK YOUR ENGLISH VOCABULARY FOR BUSINESS AND ADMINISTRATION.pdf

Ngày tải lên : 08/08/2012, 17:31

... our products and how they work, how the distribution system operates, how to deal with both suppliers and customers and how to handle complaints They also study trade and retail laws, and are accompanied ... idioms IT and e-commerce Jobs and positions Letters Meetings and presentations Money and financial issues Numbers and symbols Phrasal verbs Phrasal verbs Production and operations Recruitment 1: ... part of a , and should have 10 and the ability to 11 and inspire his or her 12 Sandra: Well, that's great! You've always got on with the people you work with, and everyone...

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Check Your English Vocabulary for Phrasal Verbs and Idioms.pdf

Ngày tải lên : 08/08/2012, 17:32

... 65 66 – 80 Idioms and other expressions using animals Idioms and other expressions for describing character and personality Idioms and other expressions using clothes Idioms and other expressions ... food and drink Phrasal verbs, idioms and other expressions using 'get' Phrasal verbs, idioms and other expressions using 'give' Phrasal verbs, idioms and other expressions using 'go' Idioms and ... effects of the drug _ after a few hours 15 Andrew and Steve have argued again Andrew and Steve have _ again 16 They refused to accept and deal with their responsibilities They refused...

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Atmel AVR Microcontroller Primer Programming and Interfaceing

Ngày tải lên : 16/08/2012, 09:03

... (Eleanore and Jackie), and thank you, Dad (Frank), for always believing in me (S.B.) Thank you, Moms (Young Shin and Rana), and thank you, Dads (Sung Bock and Chong Kon), for your encouragement and ... microcontrollers themselves are inexpensive, and the compilers and programming hardware and software are relatively inexpensive vi ATMEL AVR MICROCONTROLLER PRIMER: PROGRAMMING AND INTERFACING • • • • • The ... Jon R., Christine, Jon B., Andrew, and Graham As always, without you none of this would matter We love you! Laramie and Colorado Springs, November 2007 Steve Barrett and Daniel Pack ix Contents...

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Pro WPF and Silverlight MVVM effective Application Development

Ngày tải lên : 17/08/2012, 08:54

... ■Chapter 5: Events and Commands 111 Events 111 Events in NET 111 Events in WPF and Silverlight 112 Commands 115 Command Pattern ... introduces the ViewModel that sits between the model and view layers and mediates between the two Chapter discusses commands and events, weighing up the pros and cons of each Chapter examines various options ... formally explained the MVVM pattern and how it compared to other patterns such MVP and MVC In Chapter 1, WPF and Silverlight will be explored in some detail and their respective features highlighted...

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Install and Uninstall

Ngày tải lên : 17/08/2012, 09:44

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C# Coding Standards and Best Programming Practices

Ngày tải lên : 18/08/2012, 08:47

... http://www.dotnetspider.com/tutorials/BestPractices.aspx To develop reliable and maintainable applications, you must follow coding standards and best practices The naming conventions, coding standards and best practices described in this ... any comments to understand 9 Perform spelling check on comments and also make sure proper grammar and punctuation is used 13 Exception Handling Never a 'catch exception and nothing' If you hide ... maintainable, you (and your company) will be spending lot of time to identify issues, trying to understand code etc throughout the life of your application Purpose of coding standards and best practices...

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Open distributed automation and control with iec 61499.pdf

Ngày tải lên : 20/08/2012, 11:15

... OPEN DISTRIBUTED AUTOMATION AND CONTROL WITH IEC 61499 Open Distributed Automation and Control Open Distributed Automation and Control with IEC 61499 with IEC 61499 • • • ... DISTRIBUTED AUTOMATION AND CONTROL WITH IEC 61499 IEC 61131-3: Modern (1993) Software IEC 61131-3: Modern (1993) Software Engineering for Automation and Control Engineering for Automation and Control • ... AUTOMATION AND CONTROL WITH IEC 61499 Basic Concepts of IEC 61499 Basic Concepts of IEC 61499 • Distributed applications • Object-oriented software encapsulation and reuse • Event and data interfaces...

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Oreilly Com and .NET Component Services

Ngày tải lên : 21/08/2012, 10:53

... services, and COM+ puts a proxy and stub between the component and its client, if the client and the component instance are incompatible with any one of the services It also puts a proxy and stub ... Services Explorer and basic COM+ terminology This chapter deliberately holds your hand as you develop your first "Hello World" COM+ component Subsequent chapters much less handholding and assume you ... help you understand and accept the resulting programming model, but not bury you in the details of theory and COM+ plumbing This chapter focuses on COM+ transaction architecture and the resulting...

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Questions to .NET and Programming in C#

Ngày tải lên : 21/08/2012, 15:55

... standard expression output b) The code will compile and run d) The code will compile and but nothing will be return on run and the word “Default” the standard output will be written to the standard ... compiles and "5, Sub" is printed on the standard output b) The code compiles and "5, d) The code compiles and "2, Super" is printed on the Super" is printed on the standard output standard output ... and c) Legal at runtime, runtime b) Legal at compile time, but fails at runtime, Given these class definitions: compile and [1.5] class Superclass { } class Subclass1 extends Superclass { } and...

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Employee Turnover: Definitions and Calculations

Ngày tải lên : 21/08/2012, 17:44

... at the beginning of a period, and remain with the company at the end of the period This figure can be useful but it hides the departures of employees that joined and subsequently left during the ... improvement over the total turnover definition; retirees and employees dismissed or made redundant no longer included This definition is more precise and more relevant to internal decision-making If ... rate (including retirements, dismissals and redundancies) – for external benchmarking purposes 2) Voluntary turnover rate (excluding retirements, dismissals and redundancies) – for internal purposes...

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Architecture and the UML

Ngày tải lên : 22/08/2012, 10:37

... classes and their relationships in the logical view of a system UML modeling elements in class diagrams - Classes and their structure and behavior - Association, aggregation, dependency, and inheritance ... Collaboration diagrams -spatial representation of objects, links, and interactions Object diagrams -objects and their relationships and correspond to (simplified collaboration diagrams that not represent ... classify and extend associations, inheritance relationships, classes, and components Examples: - Class stereotypes: boundary, control, entity, utility, exception - Inheritance stereotypes: uses and...

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Tài liệu ô tô Haynes Peugeot 205 (Phần Service And Repair Manual) (2).pdf

Ngày tải lên : 23/08/2012, 10:31

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Tài liệu ô tô Haynes Peugeot 205 (Phần Service And Repair Manual).pdf

Ngày tải lên : 23/08/2012, 10:31

... further information 15 Handbrake check and adjustment Refer to Chapter 16 Underbody and fuel/brake line check 1 With the vehicle raised and supported on axle stands (see “Jacking and vehicle support”), ... nut 25 Steering and suspension check Front suspension and steering check Apply the handbrake then jack up the front of the vehicle and support it on axle stands (see “Jacking and vehicle support”) ... removal and refitting d) Oil pump - removal, inspection and refitting e) Camshaft and rocker arms - removal, inspection and refitting f) Cylinder head - removal and refitting g) Cylinder head and...

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