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2-methylacetoacetyl-coenzyme A thiolase (beta-ketothiolase) deficiency: one disease - two pathways
(2019)
Background: 2-methylacetoacetyl-coenzyme A thiolase deficiency (MATD; deficiency of mitochondrial acetoacetyl-coenzyme A thiolase T2/ “beta-ketothiolase”) is an autosomal recessive disorder of ketone body utilization and isoleucine degradation due to mutations in ACAT1.
Methods: We performed a systematic literature search for all available clinical descriptions of patients with MATD. 244 patients were identified and included in this analysis. Clinical course and biochemical data are presented and discussed.
Results: For 89.6 % of patients at least one acute metabolic decompensation was reported. Age at first symptoms ranged from 2 days to 8 years (median 12 months). More than 82% of patients presented in the first two years of life, while manifestation in the neonatal period was the exception (3.4%). 77.0% (157 of 204 patients) of patients showed normal psychomotor development without neurologic abnormalities.
Conclusion: This comprehensive data analysis provides a systematic overview on all cases with MATD identified in the literature. It demonstrates that MATD is a rather benign disorder with often favourable outcome, when compared with many other organic acidurias.
2. Änderung der Geschäftsordnung für den Senat der Fachhochschule Bonn-Rein-Sieg vom 07. April 2005
(2005)
20 Jahre Hochschul- und Kreisbibliothek Bonn-Rhein-Sieg: Eine ungewöhnliche Erfolgsgeschichte
(2018)
Als am 28. September 1998 Oberkreisdirektor Frithjof Kühn (der spätere Landrat) und Kreisdirektorin Monika Lohr für den Rhein-Sieg-Kreis sowie Gründungsrektor Prof. Dr. Hubert Severin und Kanzler Hans Stender für die Fachhochschule Rhein-Sieg (heute Hochschule Bonn-Rhein-Sieg) einen Kooperationsvertrag unterschrieben, taten sie etwas sehr Ungewöhnliches: Die wissenschaftliche Bibliothek der Fachhochschule und die öffentliche Bibliothek des Rhein-Sieg-Kreises sollten organisatorisch und räumlich zusammengelegt werden.
Innovative Technologien, veränderte Anbieterstrukturen, stets neue Leistungsangebote und wandelnde Kundenanforderungen prägen und verändern von Beginn an das Erscheinungsbild des TK-Marktes. Unter Berücksichtigung einer Betrachtung im Zeitablauf lässt sich der Markt in drei aufeinanderfolgende Phasen einteilen: 1. Marktöffnung, 2. Konsolidierung und Optimierung, 3. Automatisierung. Die Phasen sind selbstverständlich nicht Überschneidungsfrei, geben jedoch einen Hinweis auf die strukturellen Veränderungen des Marktes.
Sharing economies enabled by technical platforms have been studied regarding their economic, legal, and social effects, as well as with regard to their possible influences on CSCW topics such as work, collaboration, and trust. While a lot current research is focusing on the sharing economy and related communities, there is little work addressing the phenomenon from a socio-technical point of view. Our workshop is meant to address this gap. Building on research themes and discussion from last year’s ECSCW, we seek to engage deeper with topics such as novel socio-technical approaches for enabling sharing communities, discussing issues around digital consumer and worker protection, as well as emerging challenges and opportunities of existing platforms and approaches.
Sie sind im Bereich Qualitätsmanagement tätig und haben die Aufgabe bekommen, ein Problem systematisch zu untersuchen und methodisch zu lösen? Sie haben zu viele Aufgaben und wissen nicht, wie Sie diese priorisieren sollen? Oder haben Sie zu begrenzte Ressourcen, um alle Reklamationen gleichzeitig bearbeiten zu können? Oder wissen nicht, wie Sie einen bestimmten Prozess in seinen Grenzen zielführend verbessern können?
Nitrile-type inhibitors are known to interact with cysteine proteases in a covalent-reversible manner. The chemotype of 3-cyano-3-aza-β-amino acid derivatives was designed in which the N-cyano group is centrally arranged in the molecule to allow for interactions with the nonprimed and primed binding regions of the target enzymes. These compounds were evaluated as inhibitors of the human cysteine cathepsins K, S, B, and L. They exhibited slow-binding behavior and were found to be exceptionally potent, in particular toward cathepsin K, with second-order rate constants up to 52 900 × 103 M–1 s–1.
Background: 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency (HMGCLD) is an autosomal recessive disorder of ketogenesis and leucine degradation due to mutations in HMGCL.
Method: We performed a systematic literature search to identify all published cases. Two hundred eleven patients of whom relevant clinical data were available were included in this analysis. Clinical course, biochemical findings and mutation data are highlighted and discussed. An overview on all published HMGCL variants is provided.
Results: More than 95% of patients presented with acute metabolic decompensation. Most patients manifested within the first year of life, 42.4% already neonatally. Very few individuals remained asymptomatic. The neurologic long-term outcome was favorable with 62.6% of patients showing normal development.
Conclusion: This comprehensive data analysis provides a systematic overview on all published cases with HMGCLD including a list of all known HMGCL mutations.
Background 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency (HMGCLD) is an autosomal recessive disorder of ketogenesis and leucine degradation due to mutations in HMGCL .
Method We performed a systematic literature search to identify all published cases. 211 patients of whom relevant clinical data were available were included in this analysis. Clinical course, biochemical findings and mutation data are highlighted and discussed. An overview on all published HMGCL variants is provided.
Results More than 95% of patients presented with acute metabolic decompensation. Most patients manifested within the first year of life, 42.4% already neonatally. Very few individuals remained asymptomatic. The neurologic long-term outcome was favorable with 62.6% of patients showing normal development.
Conclusion This comprehensive data analysis provides a systematic overview on all published cases with HMGCLD including a list of all known HMGCL mutations.
3-Hydroxyisobutyrate Dehydrogenase (HIBADH) deficiency - a novel disorder of valine metabolism
(2021)
3-Hydroxyisobutyric acid (3HiB) is an intermediate in the degradation of the branched-chain amino acid valine. Disorders in valine degradation can lead to 3HiB accumulation and its excretion in the urine. This article describes the first two patients with a new metabolic disorder, 3-hydroxyisobutyrate dehydrogenase (HIBADH) deficiency, its phenotype and its treatment with a low-valine diet. The detected mutation in the HIBADH gene leads to nonsense-mediated mRNA decay of the mutant allele and to a complete loss-of-function of the enzyme. Under strict adherence to a low-valine diet a rapid decrease of 3HiB excretion in the urine was observed. Due to limited patient numbers and intrafamilial differences in phenotype with one affected and one unaffected individual, the clinical phenotype of HIBADH deficiency needs further evaluation.
In service robotics, tasks without the involvement of objects are barely applicable, like in searching, fetching or delivering tasks. Service robots are supposed to capture efficiently object related information in real world scenes while for instance considering clutter and noise, and also being flexible and scalable to memorize a large set of objects. Besides object perception tasks like object recognition where the object’s identity is analyzed, object categorization is an important visual object perception cue that associates unknown object instances based on their e.g. appearance or shape to a corresponding category. We present a pipeline from the detection of object candidates in a domestic scene over the description to the final shape categorization of detected candidates. In order to detect object related information in cluttered domestic environments an object detection method is proposed that copes with multiple plane and object occurrences like in cluttered scenes with shelves. Further a surface reconstruction method based on Growing Neural Gas (GNG) in combination with a shape distribution-based descriptor is proposed to reflect shape characteristics of object candidates. Beneficial properties provided by the GNG such as smoothing and denoising effects support a stable description of the object candidates which also leads towards a more stable learning of categories. Based on the presented descriptor a dictionary approach combined with a supervised shape learner is presented to learn prediction models of shape categories.
Experimental results, of different shapes related to domestically appearing object shape categories such as cup, can, box, bottle, bowl, plate and ball, are shown. A classification accuracy of about 90% and a sequential execution time of lesser than two seconds for the categorization of an unknown object is achieved which proves the reasonableness of the proposed system design. Additional results are shown towards object tracking and false positive handling to enhance the robustness of the categorization. Also an initial approach towards incremental shape category learning is proposed that learns a new category based on the set of previously learned shape categories.
The ability of detecting people has become a crucial subtask, especially in robotic systems which aim an application in public or domestic environments. Robots already provide their services e.g. in real home improvement markets and guide people to a desired product. In such a scenario many robot internal tasks would benefit from the knowledge of knowing the number and positions of people in the vicinity. The navigation for example could treat them as dynamical moving objects and also predict their next motion directions in order to compute a much safer path. Or the robot could specifically approach customers and offer its services. This requires to detect a person or even a group of people in a reasonable range in front of the robot. Challenges of such a real-world task are e.g. changing lightning conditions, a dynamic environment and different people shapes. In this thesis a 3D people detection approach based on point cloud data provided by the Microsoft Kinect is implemented and integrated on mobile service robot. A Top-Down/Bottom-Up segmentation is applied to increase the systems flexibility and provided the capability to the detect people even if they are partially occluded. A feature set is proposed to detect people in various pose configurations and motions using a machine learning technique. The system can detect people up to a distance of 5 meters. The experimental evaluation compared different machine learning techniques and showed that standing people can be detected with a rate of 87.29% and sitting people with 74.94% using a Random Forest classifier. Certain objects caused several false detections. To elimante those a verification is proposed which further evaluates the persons shape in the 2D space. The detection component has been implemented as s sequential (frame rate of 10 Hz) and a parallel application (frame rate of 16 Hz). Finally, the component has been embedded into complete people search task which explorates the environment, find all people and approach each detected person.