Refine
H-BRS Bibliography
- yes (577) (remove)
Departments, institutes and facilities
- Fachbereich Angewandte Naturwissenschaften (577) (remove)
Document Type
- Article (356)
- Conference Object (75)
- Part of a Book (58)
- Doctoral Thesis (26)
- Book (monograph, edited volume) (21)
- Report (20)
- Contribution to a Periodical (6)
- Preprint (5)
- Research Data (4)
- Conference Proceedings (2)
- Patent (2)
- Lecture (1)
- Book review (1)
Year of publication
Has Fulltext
- no (577) (remove)
Keywords
- GC/MS (8)
- Lehrbuch (8)
- Lignin (8)
- Chemie (6)
- Biomass (5)
- Chemometrics (5)
- Analytical pyrolysis (4)
- CD21 (4)
- Chromatography (4)
- Corrosion inhibitors (4)
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.
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.
Timely recognition of threats can be significantly supported by security assistance systems that work continuously in time and call the security personnel in case of anomalous events in the surveillance area. We describe the concept and the realization of an indoor security assistance system for real-time decision support. The system consists of a computer vision module and a person classification module. The computer vision module provides a video event analysis of the entrance region in front of the demonstrator. After entering the control corridor, the persons are tracked, classified, and potential threats are localized inside the demonstrator. Data for the person classification are provided by chemical sensors detecting hazardous materials. Due to their limited spatio-temporal resolution, a single chemical sensor cannot localize this material and associate it with a person. We compensate this deficiency by fusing the output of multiple, distributed chemical sensors with kinematical data from laser-range scanners. Considering both the computer vision formation and the results of the person classification affords the localization of threats and a timely reaction of the security personnel.
Als rohstoffarme und exportorientierte Wirtschaftsnation ist die Bundesrepublik in ho- hem Maß auf die Sicherung und Sicherheit der Logistikketten im grenzüberschreiten- den Verkehr angewiesen. Angesichts der komplexen Transportstrukturen bei grenz- überschreitenden Transporten kommt den eingesetzten Kontroll- und Prüfverfahren besondere Bedeutung zu: Einerseits müssen Kostenbelastungen, Unterbrechungen und Verzögerungen in der Transportkette minimiert, andererseits besonders illegale Einfuhren, Transporte und Substanzen unterbunden werden. Von besonderer Bedeu- tung für Verdachts- bzw. Stichprobenkontrollen ist der Einsatz speziell trainierter Spür- hunde. Als besonders leistungsfähige ‚lebende Sensoren‘ sind sie in der Lage, eine Vielzahl von Stoffen zu detektieren. Der Einsatz von Spürhunden unterliegt allerdings engen Grenzen: Hoher Trainingsaufwand, eng begrenzte Einsatzdauer, begrenzte Verfügbarkeit. Die Entwicklung neuer, optimierter Einsatzverfahren für Spürhunde z. B. mit höheren Durchsatzraten und überprüfbarer Verlässlichkeit durch Einbindung technischer Systeme ist daher ein wichtiger Beitrag für die Sicherung und Sicherheit der Logistikketten.