Lysosomal Storage Disorders: A Practical Guide
Language: English
Published by John Wiley & Sons, 2022
- Hardcover
- New
Condition: New
US$ 159.77
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Add to basketItem description from seller
The EditorsAtul B. Mehta is Emeritus Professor at University College London. He trained in Medicine at Cambridge University and King s College London completed his doctorate at Imperial College London and works as a haematologist and physician in London. H.
Seller Inventory # 578960970
- Title
- Lysosomal Storage Disorders: A Practical Guide
- Author
- AB Mehta
- Publisher
- John Wiley & Sons
- Publication year
- 2022
- Condition
- New
- Binding
- Gebunden
- Language
- English
- ISBN 10
- 111969728X
- ISBN 13
- 9781119697282
- Edition
- 2nd Edition
Lysosomal Storage Disorders
Enables readers to gain both holistic and specific knowledge on the topic of Lysosomal Storage Disorders
In the past few decades, we have witnessed a ‘golden age’ of Lysosomal Storage Disorders (LSDs) clinical care, diagnosis, and research and this book is a well-timed review of the rapidly developing subject of LSDs. Contributions from many leading scientists and clinicians in the field provide the reader with a completely comprehensive overview of the subject. Topics covered in the book include:
- The general aspects of LSDs, with special attention paid to physiology and pathology
- Clinical and laboratory diagnosis, including newborn screening and the genetics of LSDs
- Individual LSDs, such as the various sphingolipidoses, mucopolysaccharidoses, glycogen storage disease, glycoproteinoses, galactosialidosis, and neuronal ceroid lipofuscinoses
- Other disorders of the lysosome, including those involving defects in the lysosomal membrane and in the emerging roles of the lysosome in cellular metabolism
- The existing and emerging treatments for LSDs
- Key patient issues such as availability and disease awareness, including in the Third World.
For researchers and industry professionals in the field of LSDs, this book serves as a completely comprehensive reference work to understand both theoretical concepts and how they may be used in practical applications. For students of LSDs, it provides a solid base of foundational knowledge and serves as a practical guide.
"Synopsis" may belong to another edition of this title.
About the Author
The Editors
Atul B. Mehta is Emeritus Professor at University College London. He trained in Medicine at Cambridge University and King’s College London; completed his doctorate at Imperial College London and works as a haematologist and physician in London. He was the Founder and Director of the Lysosomal Storage Disorders Centre at the Royal Free, which grew to be the largest centre in the UK and is one of the foremost clinical academic centres for LSDs in the world. His goal has always been to provide patient-centred care as part of a coordinated team of professionals. He now works in private practice, charitable, educational and research roles, including consultancy.
Bryan Winchester is Emeritus Professor of Biochemistry at the UCL Great Ormond Street Institute of Child Health, University College London. After studying Chemistry at Cambridge University, he obtained a Ph.D. in Biochemistry at University College London. He taught Biochemistry and carried out research on lysosomal storage diseases at London University for over 40 years. From 1988–2006, he worked closely with the Enzyme Diagnostic Laboratory at Great Ormond Street Hospital and was chairman of the European Study Group on Lysosomal Diseases from 1997–2007. On retirement he became a Trustee of the British MPS Society.
"About the title" may belong to another edition of this title.
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Moluna GmbH
Engberdingdamm 27
Greven, Germany 48268
Terms of sale
Instructions for revocation/
Standard Business Terms and customer information/ data protection declaration
Revocation right for consumers
(A ‘consumer’ is any natural person who concludes a legal transaction which, to an overwhelming extent, cannot be attributed to either his commercial or independent professional activities.)
Instructions for revocation
Revocation right
You have the right to revoke this contract within one month without specifying any reasons.
The revocation period is one month with effect from the day,
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on which you or a third party nominated by you, which is not the carrier, had taken possession of the products, provided you had ordered one or more products within the scope of a standard order and this/these product/products is/are delivered uniformly;
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on which you or a third party nominated by you, which is not the carrier, had taken possession of the last product, provided you had ordered several products within the scope of a standard order and these products are delivered separately;
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on which you or a third party nominated by you, which is not the carrier, had taken possession of the last part delivery or the last unit, provided you had ordered a product, which is delivered in several part deliveries or units;
In order to exercise your revocation right, you must inform us (Moluna GmbH, Engberdingdamm 27, 48268 Greven, Telephone number: 02571/5 69 89 33, Fax number: 02571/5 69 89 30, E-Mail address: abe@moluna.de) of your decision to revoke this contract by means of a clear declaration (e.g. a letter sent via post, fax or email). You can use the enclosed specimen revocation form for this, which however is not mandatory.
In order to safeguard the revocation period, it is sufficient that you send the notification about the exercise of the revocation right before the expiry of the revocation period.
Consequences of the revocation
If you revoke this contract, we shall repay all the payments, which we received from you, including the delivery costs (with the exception of additional costs, which arise from that fact that you selected a form of delivery other than the most reasonable standard delivery offered by us), immediately and at the latest within 14 days from the day on which we received the notification about the revocation of this contract from you. We use the same means of payment, which you had originally used during the original transaction, for this repayment unless expressly agreed otherwise with you; you will not be charged any fees owing to this repayment.
We can refuse the repayment until the products are returned to us or until you have furnished evidence that you have sent the products back to us, depending on whichever is earlier.
You must return or transfer the products to us immediately and, in any case, at the latest within 14 days with effect from the day on which you inform us of the revocation of this contract. The deadline is maintained if you send the products before the expiry of the 14 day deadline.
You bear the direct costs for returning the products.
You must pay for any depreciation of the products only if this depreciation can be attributed to any handling with you that was not necessary for checking the condition, features and functionality of the products.
Criteria for exclusion or expiry
The revocation right is not available for contracts
for delivery of products, which are not prefabricated and for whose manufacturing an individual selection or stipulation by the consumer is important or which are clearly tailored to the personal requirements of the consumer;
for delivery of products, which can spoil quickly or whose use-by date would be exceeded quickly;
for delivery of alcoholic drinks, whose price was agreed at the time of concluding the contract, which however can be delivered 30 days after the conclusion of the contract at the earliest and whose current value depends on the fluctuations in the market, on which the entrepreneur has no influence;
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Specimen - revocation form
(If you wish to revoke the contract, please fill up this form and send it back to us.)
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To Moluna GmbH, Münsterstr. 105, 48268 Greven, Fax number: 02571/5 69 89 30, Email address: abe@moluna.de :
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I/we () herewith revoke the contract concluded by me/ us () regarding the purchase of the following products ()/
the provision of the following service () -
Ordered on ()/ received on ()
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Name of the consumer(s)
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Address of the consumer(s)
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Standard Business Terms and customer information / data protection declaration
I. Standard business terms
§ 1 Basic provisions
(1) The following terms and conditions of business apply for all contracts concluded with us as the supplier (Moluna GmbH) via the websites AbeBooks and/or ZVAB. Unless otherwise agreed, the inclusion of your own terms and conditions is explicitly rejected.
(2) A ‘consumer’ in the sense of the following regulations is every natural person who concludes a legal transaction which, to an overwhelming extent, cannot be attributed to either his commercial or independent professional activities. The term ‘businessman’ refers to every natural person, legal person or legally responsible partnership that concludes a legal transaction in pursuance of his/its independent professional or commercial activity.
§ 2 Conclusion of the contract
(1) The subject-matter of the contract is the selling of products .
(2) If an article is placed by us with AbeBooks or ZVAB, the activation of the page on AbeBooks or ZVAB shall involve the binding offer to conclude a contract under the terms and conditions contained in the article page.
(3) The contract shall become effective via the online shopping cart system as follows:
The products intended for purchase are moved to the "shopping cart". You can select the "Shopping Cart" using the appropriate buttons on the navigation bar and make changes there at any time.
After calling up the “Checkout” page and entering the required personal data and payment and shipping conditions, all order information is then displayed again on the order summary page.
Before submitting the order, you have the ability once more to review or change any information here (you may also use the "back" button on the Internet browser), or to cancel the purchase.
By clicking the “Buy now” button to submit the order, you declare your legally binding acceptance of the order which makes the contract effective.
(4) The execution of the order and the sending of all the details necessitated by the conclusion of the contract take place via e-mail, in a partially-automated manner. Consequently, you have to ensure that the e-mail address that you have deposited with us is the correct one, and that the receipt of the respective e-mails is guaranteed. In particular, you have to ensure that the respective e-mails are not blocked by a SPAM filter.
§ 3 Right of retention, reservation of proprietary rights
(1) You can only exercise a right of retention if the situation in question involves claims arising from the same contractual relationship.
(2) The goods remain our property until the purchase price is paid in full.
(3) If you are a businessman, the following conditions also apply:
a) We retain ownership of the goods until all the claims arising from the ongoing business relationship have been settled in full. The goods subject to retention of title may not be pledged or transferred by way of security before ownership of the said goods changes hands.
b) You can re-sell the goods within the framework of an orderly transaction. In this regard, you hereby cede all the claims amounting to the magnitude of the billing amount that accrue to you as a result of the re-selling operation to us, and we accept the cession. Furthermore, you are authorised to collect the claim in question. However, insofar as you do not discharge your payment obligations in an orderly fashion, we reserve the right to collect the claim ourselves.
c) In a situation involving the combination and amalgamation of the goods subject to retention of title, we acquire co-ownership of the newly-formed item. This co-ownership corresponds to the ratio that exists between the invoice value of the goods subject to retention of title and the other processed items at the time of processing.
d) If you make a request of this nature, we shall be obligated to release the securities that are due to us, to the extent that the realisable value of our securities exceeds the claim to be secured by more than 10%. We are responsible for selecting the securities to be released.
§ 4 Warranty
(1) The statutory warranty rights are applicable.
(2) As a consumer, you are requested to promptly check the product for completeness, visible defects and transport damage as soon as it is delivered, and promptly disclose your complaints to us and the shipping company in writing. Even if you do not comply with this request, it shall have no effect on your legal warranty claims.
(3) If a characteristic of the goods deviates from the objective requirements, the deviation shall only be deemed to be agreed if you were informed of the same by us before submitting the contractual declaration and the deviation was expressly and separately agreed between the contracting parties.
(4)
Insofar as you are an entrepreneur, the following shall apply in deviation from the above warranty provisions:
a) Only our own specifications and the manufacturer's product description shall be deemed agreed as the quality of the goods, but not other advertising, public promotions and statements by the manufacturer.
b) In the event of defects, we shall, at our discretion, provide warranty by rectification of the defect or subsequent delivery. If the rectification of defects fails, you may, at your option, demand a reduction in price or withdraw from the contract. The rectification of defects shall be deemed to have failed after a second unsuccessful attempt, unless the nature of the goods or the defect or other circumstances indicate otherwise. In the event of rectification of defects, we shall not be obliged to bear the increased costs arising from the transport of the goods to a place other than the place of performance, unless such transport is in accordance with the intended use of the goods.
c) The warranty period shall be one year from delivery of the goods. The shortening of the period shall not apply
- for culpable damage attributable to us arising from injury to life, limb or health and for other damage caused intentionally or by gross negligence;
- insofar as we have fraudulently concealed the defect or have assumed a guarantee for the quality of the item;
- in the case of items which have been used for a building in accordance with their customary use and have caused its defectiveness;
- in the case of statutory rights of recourse that you may assert against us in connection with rights arising from defects.
§ 5 Choice of law, place of fulfilment, jurisdiction
(1) German law shall apply. This choice of law only applies to customers if it does not result in the revocation of the protection guaranteed by the mandatory provisions of the law of the country in which the respective customer’s usual place of residence is located (benefit-of-the-doubt principle).
(2) If you are not a consumer, but a businessman, a legal entity under public law or an institutional fund governed by public law, our place of business is the place of jurisdiction as well as the place of fulfilment for all services that follow from the business relationships that exist with us. The same condition applies to situations in which you are not associated with a general place of jurisdiction in Germany or the EU, as well as situations in which the place of residence or the usual place of residence is not known at the time of commencement of proceedings. This has no bearing on the capacity to call upon the court associated with another place of jurisdiction.
(3) The provisions of the UN Convention on Contracts for the International Sale of Goods are explicitly inapplicable.
II. Customer information
- Identity of the seller
Moluna GmbH
Engberdingdamm 27
48268 Greven
Germany
Telephone: 02571/5698933
E-Mail: abe@moluna.de
- Information regarding the conclusion of the contract
The technical steps associated with the conclusion of the contract, the contract conclusion itself and the correction options are executed in accordance to the regulations "conclusion of the contract" in our standard business terms (part I.).
- Contractual language, saving the text of the contract
3.1 Contract language shall be English.
3.2 The complete text of the contract is not saved with us. Before the order is sent, the contract data can be printed out or electronically saved using the browser’s print function. After the order is received by us, the order data, the legally-mandated details related to distance selling contracts and the standard business terms are re-sent to you via e-mail.
- Main features of the product or service
The key features of the goods and/or services can be found in the respective quote.
- Prices and payment arrangements
5.1 The prices mentioned in the respective offers represent total prices, as do the shipping costs. They include all the price components, including all the incidental taxes.
5.2 The dispatch costs that are incurred are not included in the purchase price. They can be viewed by clicking the appropriate button on our website or in the respective quote, are shown separately over the course of the order transaction and must additionally be borne by you, insofar as free delivery is not confirmed.
5.3 The payment methods that are available to you are shown by clicking the appropriate button on our website or are disclosed in the respective quote.
5.4 Unless otherwise specified for the respective payment methods, the payment claims arising from the contract that has been concluded become payable immediately.
- Delivery conditions
6.1 The delivery conditions, delivery date and existing supply restrictions, if applicable, can be found by clicking the appropriate button on our website or in the respective quote.
Unless a different period is specified in the item description or our delivery conditions, the goods are delivered within 3-5 days after the conclusion of the contract (in case an advance payment has been agreed upon, after the payment authorisation).
6.2 If you are a consumer, the following is statutorily regulated: The risk of the sold item accidentally being destroyed or degraded during shipping only passes over to you when the item in question is delivered, regardless of whether or not the shipping operation is insured. This condition does not apply if you have independently commissioned a transport company that has not been specified by us or a person who has otherwise been appointed to execute the shipping operation.
If you are a businessman, the delivery and shipping operations take place at your own risk.
- Statutory warranty right
Liability for defects is governed by the “Warranty” provisions in our General Terms and Conditions of Business (Part I).
last update: 01.01.2022
Data protection declaration
Unless stated otherwise below, the provision of your personal data is neither legally nor contractually obligatory, nor required for conclusion of a contract. You are not obliged to provide your data. Not providing it will have no consequences. This only applies as long as the processing procedures below do not state otherwise.
“Personal data” is any information relating to an identified or identifiable natural person.
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Contact us at any time. The contact details of the person responsible for data processing can be found in our legal notice.
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If the data processing outlined here is based on our legitimate interests in accordance with Article 6(1)f) GDPR, you have the right for reasons arising from your particular situation to object at any time to the processing of your data with future effect.
If the objection is successful, we will no longer process the personal data, unless we can demonstrate compelling legitimate grounds for the processing that outweigh your interests or rights and freedoms, or the processing is intended for the assertion, exercise or defence of legal claims.
last update: 10.01.2022