ETF-QO Mutants Uncoupled Fatty Acid β-Oxidation and Mitochondrial Bioenergetics Leading to Lipid Pathology.

The electron-transfer flavoprotein dehydrogenase gene () that encodes the ETF-ubiquinone oxidoreductase (ETF-QO) has been reported to be the major cause of multiple acyl-CoA dehydrogenase deficiency (MADD). ETF-QO is an electron carrier that mainly functions in mitochondrial fatty acid β-oxidation and the delivery of electrons to the ubiquinone pool in the mitochondrial respiratory chain. A high frequency of c.

Coenzyme Q10 serves to couple mitochondrial oxidative phosphorylation and fatty acid β-oxidation, and attenuates NLRP3 inflammasome activation.

Multiple acyl-CoA dehydrogenase deficiency (MADD), an autosomal recessive metabolic disorder of fatty acid metabolism, is mostly caused by mutations in the ETFA, ETFB or ETFDH genes that result in dysfunctions in electron transfer flavoprotein (ETF) or electron transfer flavoprotein-ubiquinone dehydrogenase (ETFDH). In β-oxidation, fatty acids are processed to generate acyl-CoA, which is oxidised by flavin adenine dinucleotide and transfers an electron to ETF and, through ETFDH, to mitochondrial respiratory complex III to trigger ATP synthesis. Coenzyme Q10 (CoQ10) is believed to be a potential treatment that produces symptom relief in some MADD patients.

Kinetically Enhanced Approach for Rapid and Tunable Self-Assembly of Rod-Coil Block Copolymers.

A facile approach is reported to process rod-coil block copolymers (BCPs) into highly ordered nanostructures in a rapid, low-energy process. By introducing a selective plasticizer into the rod-coil BCPs during annealing, both the annealing temperature and time to achieve thermodynamic equilibrium and highly ordered structures can be decreased. This process improvement is attributed to enhanced chain mobility, reduced rod-rod interaction, and decreased rod-coil interaction from the additive.

The proportion of self-rated olfactory dysfunction does not change across the life span.

Background: Previous reports indicated that self-reports of olfactory function are unreliable. The occurrence of measured olfactory dysfunction is approximately 20% in the general population and consistently higher than that of self-reported olfactory dysfunction. To further understand the frequencies of self-rated olfactory function in different age groups and its relation with measured olfactory function, a prospective investigation of self-rated and measured olfactory function was performed.

Learning curve of septomeatoplasty.

Objective: To determine the time for us to train a well-trained surgeon to perform septomeatoplasty.

Material And Methods: From July 1, 2003 to June 30, 2007, we included 75 patients with nasal septal deviation and chronic hypertrophic rhinitis received septomeatoplasty into this study. All the procedures were performed by four surgeons trained in the same tertiary referral center.

Survey of the frequency of olfactory dysfunction in Taiwan.

Background: Olfactory function is related to the scrutiny of environmental dangers and the tasting of food. However, olfactory dysfunction is not as distinctive as visual loss and may go unnoticed, especially when olfactory function deteriorates slowly. Most studies have used either questionnaires or relatively insensitive tests to assess olfactory dysfunction.

Cross-cultural application of the "Sniffin' Sticks" odor identification test.

Background: Odor identification tasks are an important tool for the clinical assessment of olfactory function. However, cultural differences prevent odor identification tests from being used in different countries, because odor identification is strongly dependent on familiarity with these odors. The "Sniffin' Sticks" olfactory test has been developed in Europe and is used for assessment of olfactory function in many European countries.

CO2 laser myringotomy in children with otitis media with effusion.

Background: The study aimed to evaluate the clinical application of CO2 laser myringotomy in children with otitis media with effusion (OME) under topical anaesthesia in an office setting.

Methods: Laser myringotomy was performed with the CO2 laser Otoscan (OtoLAM) in 54 children (73 ears) with OME. The procedure on the tympanic membrane was performed under topical anaesthesia using Bonain's solution or 10 per cent Xylocaine (lidocaine) solution for 30 minutes before surgery.