Search

The main objective of the paper is to give an overview of prerequisites and enablers for individualized production from the perspective of flexible, agile and sustainable production of customized and personalized products. Increasing volatility in the global and local markets, shortening innovation and product life cycles, as well as a tremendously increasing number of variants, call for production facilities and networks and operations management which comply with these changing demands. The paper presents a set of developed concepts, methods and tools based on the recognized needs of manufacturing companies. The observed manufacturing domain is characterized with highly customized and personalized products produced in competitively in a high-cost country with short delivery times and high quality requirements. Micro and desktop factories can be seen as one type of solution to point-of-need manufacturing of customized and personalized products, such as hand-held consumer electronics or medical implants. The LeanMES concept and associated solution blocks aim for more efficient operation via digital tools. The Competitive Sustainable Manufacturing Hotel (CSM-Hotel) concept of a platform targeted for SMEs collaborating under same factory roof or in the same area. The A-NET vision offers a new holistic framework for industrial supply networks to manage development more agilely and for deeper strategic cooperation. [ABSTRACT FROM AUTHOR]

Copyright of Somnologie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Somnologie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

An expanded polyglutamine domain in the TATA-binding protein (TBP) has been described in patients with spinocerebellar ataxia type 17 (SCA17) characterized by cerebellar ataxia associated with dementia. TBP is a general transcription initiation factor that regulates the expression of most eukaryotic genes transcribed by RNA polymerase II. SCA17, as an autosomal dominantly inherited progressive neurodegenerative disorder, is caused by heterozygous expansion of a CAG repeat coding for glutamine. Alleles with 27 to a maximum of 44 glutamine residues were found as the normal range, whereas expansions above 45 repeat units were considered pathological. Here, we present a patient with a very severe phenotype with a late onset but rapidly progressing ataxia associated with dementia and homozygous 47 glutamine residues caused by an apparent partial isodisomy 6. This extraordinary case has important implications for the insights of TBP and SCA17. The expanded polyglutamine domain in both TBP copies is not correlated with embryonic death indicating that the normal function of the protein is not disrupted by this kind of mutation but may account for the dementia seen in this patient. [ABSTRACT FROM AUTHOR]